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Corsi DR, Kelly B, Nair N, Luo M, Osler B, Cho SH, Mehrotra P, Wiener D, Johnson D. Clinical Variability in Presentation and Management of Quadricuspid Aortic Valve: A Case Series. CASE (PHILADELPHIA, PA.) 2025; 9:130-134. [PMID: 40309471 PMCID: PMC12038186 DOI: 10.1016/j.case.2024.12.008] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Indexed: 05/02/2025]
Abstract
•Rare presentation of three QAV cases with varied clinical manifestations is reported. •Multimodal imaging is crucial for accurate QAV diagnosis and classification. •QAV is often associated with aortic regurgitation requiring intervention. •Surgical repair is preferred over replacement in suitable patients with QAV. •Long-term follow-up is essential for patients with QAV, even if asymptomatic.
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Affiliation(s)
- Douglas R. Corsi
- Sidney Kimmel Medical College at Thomas Jefferson University, Philadelphia, Pennsylvania
- Department of Internal Medicine at Rutgers Robert Wood Johnson University Hospital, New Brunswick, New Jersey
| | - Brooke Kelly
- Sidney Kimmel Medical College at Thomas Jefferson University, Philadelphia, Pennsylvania
| | - Nikita Nair
- Sidney Kimmel Medical College at Thomas Jefferson University, Philadelphia, Pennsylvania
| | - Meiqi Luo
- Sidney Kimmel Medical College at Thomas Jefferson University, Philadelphia, Pennsylvania
| | - Brian Osler
- Division of Cardiology, Thomas Jefferson University Hospital, Philadelphia, Pennsylvania
| | - Sung-Hae Cho
- Division of Cardiology, Thomas Jefferson University Hospital, Philadelphia, Pennsylvania
| | - Praveen Mehrotra
- Division of Cardiology, Thomas Jefferson University Hospital, Philadelphia, Pennsylvania
| | - David Wiener
- Division of Cardiology, Thomas Jefferson University Hospital, Philadelphia, Pennsylvania
| | - Drew Johnson
- Division of Cardiology, Thomas Jefferson University Hospital, Philadelphia, Pennsylvania
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2
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Caivano D, Cicogna M, Orvieto S, Spitale D, Porciello F. Quadricuspid aortic valve in a 16-year-old Quarter horse. J Equine Vet Sci 2024; 142:105199. [PMID: 39306145 DOI: 10.1016/j.jevs.2024.105199] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/29/2023] [Revised: 05/20/2024] [Accepted: 09/17/2024] [Indexed: 09/27/2024]
Abstract
A 16-year-old horse, 400 kg, male, Quarter horse gelding was examined for a cardiac murmur that had not been previously heard. Physical examination revealed a regularly irregular pulse and a grade III/VI, decrescendo, diastolic murmur with a point of maximum intensity over the left heart base. Base-apex standard electrocardiographic examination at rest showed sinus rhythm with second-degree atrio-ventricular blocks. Echocardiography identified the presence of four aortic valve cusps, two equal larger and two unequal smaller cusps. Color flow Doppler examination showed a diastolic regurgitant jet emerging from the central region of closed aortic valve. Based on clinical and echocardiographic findings a diagnosis of mild to moderate aortic valve insufficiency secondary to quadricuspid aortic valve was made. This report describes a rare congenital heart defect that can be detected by transthoracic echocardiography in the horse.
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Affiliation(s)
- D Caivano
- Department of Veterinary Medicine, University of Perugia, Via San Costanzo 4, Perugia, 06126, Italy.
| | - M Cicogna
- Department of Veterinary Medicine, University of Perugia, Via San Costanzo 4, Perugia, 06126, Italy
| | - S Orvieto
- Private Practitioner, Perugia, Italy
| | - D Spitale
- Department of Veterinary Medicine, University of Perugia, Via San Costanzo 4, Perugia, 06126, Italy
| | - F Porciello
- Department of Veterinary Medicine, University of Perugia, Via San Costanzo 4, Perugia, 06126, Italy
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Choi P, Paulsen M, Lin Y, Moskalik W, Ji A, Jackson E, Malik S, Burton E, Woo YJ, Burdon T. Uncommon presentations of type A quadricuspid aortic valve in the Septuagenarian. J Cardiothorac Surg 2024; 19:301. [PMID: 38812010 PMCID: PMC11134947 DOI: 10.1186/s13019-024-02696-w] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/17/2023] [Accepted: 03/24/2024] [Indexed: 05/31/2024] Open
Abstract
BACKGROUND Quadricuspid aortic valve (QAV) is a rare congenital anomaly characterized by the presence of four cusps instead of the usual three. It is estimated to occur in less than 0.05% of the population, with Type A (four equal-sized leaflets) accounting for roughly 30% of QAV subtypes. Based on limited clinical series, the usual presentation is progressive aortic valve regurgitation (AR) with symptoms occurring in the fourth to sixth decade of life. Severe aortic valve stenosis (AS) and acute AR are very uncommon. CASE PRESENTATION We describe two cases of Type A QAV in patients who remained asymptomatic until their seventies with very uncommon presentations: one with severe AS and one with acute, severe AR and flail leaflet. In Case A, a 72-year-old patient with history of moderate AS presents to clinic with progressive exertional dyspnea. During work-up for transcatheter vs. surgical replacement pre-operative computed tomography angiogram (CTA) reveals a quadricuspid aortic valve with severe AS, and the patient undergoes surgical aortic valve replacement. Pre-discharge transthoracic echocardiography (TTE) shows good prosthetic valve function with no gradient or regurgitation. In Case B, a 76-year-old patient is intubated upon arrival to the hospital for acute desaturation, found to have wide open AR on catheterization, and transferred for emergent intervention. Intraoperative TEE reveals QAV with flail leaflet and severe AR. Repair is considered but deferred ultimately due to emergent nature. Post-operative TTE demonstrates good prosthetic valve function with no regurgitation and normal biventricular function. CONCLUSIONS QAV can present as progressive severe AS and acute AR, with symptoms first occurring in the seventh decade of life. The optimal treatment for QAV remains uncertain. Although aortic valve repair or transcatheter option may be feasible in some patients, aortic valve replacement remains a tenable option.
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Affiliation(s)
- Perry Choi
- Department of Cardiothoracic Surgery, Stanford University Medical Center, 300 Pasteur Drive Stanford, Stanford, CA, 94305, USA
- Department of Cardiac Surgery, VA Palo Alto Health Care System, Palo Alto, CA, USA
| | - Michael Paulsen
- Department of Cardiothoracic Surgery, Stanford University Medical Center, 300 Pasteur Drive Stanford, Stanford, CA, 94305, USA
| | - Yihan Lin
- Department of Cardiothoracic Surgery, Stanford University Medical Center, 300 Pasteur Drive Stanford, Stanford, CA, 94305, USA
| | - William Moskalik
- Department of Cardiac Surgery, VA Palo Alto Health Care System, Palo Alto, CA, USA
| | - Angela Ji
- Department of Anesthesiology, VA Palo Alto Health Care System, Palo Alto, CA, USA
| | - Ethan Jackson
- Department of Anesthesiology, Stanford University Medical Center, Palo Alto, CA, USA
| | - Sachin Malik
- Department of Radiology, Stanford University Medical Center, Palo Alto, CA, USA
| | - Elan Burton
- Department of Cardiothoracic Surgery, Stanford University Medical Center, 300 Pasteur Drive Stanford, Stanford, CA, 94305, USA
- Department of Cardiac Surgery, VA Palo Alto Health Care System, Palo Alto, CA, USA
| | - Y Joseph Woo
- Department of Cardiothoracic Surgery, Stanford University Medical Center, 300 Pasteur Drive Stanford, Stanford, CA, 94305, USA
| | - Thomas Burdon
- Department of Cardiothoracic Surgery, Stanford University Medical Center, 300 Pasteur Drive Stanford, Stanford, CA, 94305, USA.
- Department of Cardiac Surgery, VA Palo Alto Health Care System, Palo Alto, CA, USA.
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Khatun N, Kaliounji A, Alkoutami SS, Francois J, John S. Quadricuspid Aortic Valve: An Incidental Finding in an Elderly Man. Cureus 2023; 15:e39536. [PMID: 37366439 PMCID: PMC10290899 DOI: 10.7759/cureus.39536] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 05/26/2023] [Indexed: 06/28/2023] Open
Abstract
Quadricuspid aortic valve (QAV) is a very rare congenital abnormality. Here, we present a rare case of QAV incidentally noted in a patient at an advanced age during transthoracic echocardiography (TTE). A 73-year-old man with a history of hypertension, hyperlipidemia, diabetes, and treated prostate cancer was admitted to the hospital with palpitations. An electrocardiogram (ECG) showed T-wave inversion in V5-V6, with initial troponin levels mildly elevated. Acute coronary syndrome was ruled out by serial ECGs that were unchanged, and troponins downtrended. TTE showed a rare and incidental finding of type A QAV with four equal cusps with mild aortic regurgitation.
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Affiliation(s)
- Nazima Khatun
- Internal Medicine, State University of New York (SUNY) Downstate Medical Center, Brooklyn, USA
| | - Aboud Kaliounji
- Internal Medicine, State University of New York (SUNY) Downstate Medical Center, Brooklyn, USA
| | - Sami S Alkoutami
- Internal Medicine, St. George's University School of Medicine, St. George's, USA
| | - Jonathan Francois
- Cardiology, State University of New York (SUNY) Downstate Medical Center, Brooklyn, USA
| | - Sabu John
- Cardiology, Kings County Hospital Center, Brooklyn, USA
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5
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Beddingfield RH, Rashid ZA, Pagel PS. A Five-Leaf Clover or an Exceptionally Rare Cause of Severe Aortic Insufficiency? J Cardiothorac Vasc Anesth 2022; 36:4534-4537. [PMID: 36038442 DOI: 10.1053/j.jvca.2022.07.029] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/26/2022] [Accepted: 07/27/2022] [Indexed: 11/11/2022]
Affiliation(s)
| | - Zahir A Rashid
- Department of Cardiothoracic Surgery, SSM-St. Mary's Hospital, Madison, WI
| | - Paul S Pagel
- Anesthesia Service, Clement J. Zablocki Veterans Affairs Medical Center, Milwaukee, WI.
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Sopek Merkaš I, Lakušić N, Paar MH. Quadricuspid aortic valve and right ventricular type of myocardial bridging in an asymptomatic middle-aged woman: A case report. World J Clin Cases 2022; 10:8954-8961. [PMID: 36157661 PMCID: PMC9477056 DOI: 10.12998/wjcc.v10.i25.8954] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/23/2022] [Revised: 05/19/2022] [Accepted: 07/27/2022] [Indexed: 02/05/2023] Open
Abstract
BACKGROUND Quadricuspid aortic valve (QAV) is a very rare congenital cardiac defect with the incidence of 0.0125%-0.033% (< 0.05%) predominantly causing aortic regurgitation. A certain number of patients (nearly one-half) have abnormal function and often require surgery, commonly in their fifth or sixth decade. QAV usually appears as an isolated anomaly but may also be associated with other cardiac congenital defects. Echocardiography is considered the main diagnostic method although more and more importance is given to computed tomography (CT) and magnetic resonance imaging (MRI) as complementary methods. CASE SUMMARY A 60-year-old female patient was referred for transthoracic ultrasound of the heart as part of a routine examination in the treatment of arterial hypertension. She did not have any significant symptoms. QAV was confirmed and there were no elements of valve stenosis with moderate aortic regurgitation. At first, it seemed that in the projection of the presumed left coronary cusp, there were two smaller and equally large cusps along with two larger and normally developed cusps. Cardiac CT imaging was performed to obtain an even more precise valve morphology and it showed that the location of the supernumerary cusp is between the right and left coronary cusp, with visible central malcoaptation of the cusps. Also, coronary computed angiography confirmed the right-type of myocardial bridging at the distal segment of the left anterior descending coronary artery. Significant valve dysfunction often occurs in middle-aged patients and results in surgical treatment, therefore, a 1-year transthoracic echocardiogram control examination and follow-up was recommended to our patient. CONCLUSION This case highlights the importance of diagnosing QAV since it leads to progressive valve dysfunction and can be associated with other congenital heart defects which is important to detect, emphasizing the role of cardiac CT and MRI.
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Affiliation(s)
- Ivana Sopek Merkaš
- Department of Cardiology, Special Hospital for Medical Rehabilitation Krapinske Toplice, Krapinske Toplice 49217, Croatia
| | - Nenad Lakušić
- Department of Cardiology, Special Hospital for Medical Rehabilitation Krapinske Toplice, Krapinske Toplice 49217, Croatia
- Department of Clinical Medicine, Faculty of Dental Medicine and Health Osijek, Osijek 31000, Croatia
- Department of Internal Medicine, Family Medicine and History of Medicine, Faculty of Medicine Osijek, Osjiek 31000, Croatia
| | - Maja Hrabak Paar
- Department of Diagnostic and Interventional Radiology, University Hospital Centre Zagreb, Zagreb 10000, Croatia
- School of Medicine, University of Zagreb, Zagreb 10000, Croatia
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7
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Blazekovic R, Catic J, Barisin S, Kusurin M, Gjorgjievska S, Glumbic S. Combined Disease of the Quadricuspid Aortic Valve. Tex Heart Inst J 2022; 49:487382. [DOI: 10.14503/thij-19-7177] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/07/2022]
Affiliation(s)
- Robert Blazekovic
- 1 Department of Cardiac and Transplant Surgery, University Hospital Dubrava, Zagreb, Croatia
- 2 Faculty of Medicine, Josip Juraj Strossmayer University of Osijek, Osijek, Croatia
| | - Jasmina Catic
- 2 Faculty of Medicine, Josip Juraj Strossmayer University of Osijek, Osijek, Croatia
- 3 Department of Cardiology, University Hospital Dubrava, Zagreb, Croatia
| | - Stjepan Barisin
- 2 Faculty of Medicine, Josip Juraj Strossmayer University of Osijek, Osijek, Croatia
- 4 Department of Anesthesiology, University Hospital Osijek, Osijek, Croatia
| | - Marko Kusurin
- 1 Department of Cardiac and Transplant Surgery, University Hospital Dubrava, Zagreb, Croatia
| | - Savica Gjorgjievska
- 1 Department of Cardiac and Transplant Surgery, University Hospital Dubrava, Zagreb, Croatia
| | - Stanislav Glumbic
- 5 Department of Internal Medicine, General Hospital Virovitica, Virovitica, Croatia
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8
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Válvula aórtica cuadricúspide. Presentación de dos casos. CIRUGIA CARDIOVASCULAR 2022. [DOI: 10.1016/j.circv.2022.01.001] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/19/2022] Open
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Sohn J, Arain FD. Two Consecutive Cases of Quadricuspid Aortic Valve and a Review of 149 Cases. J Cardiothorac Vasc Anesth 2021; 36:717-723. [PMID: 33731298 DOI: 10.1053/j.jvca.2021.02.033] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/09/2020] [Revised: 01/29/2021] [Accepted: 02/09/2021] [Indexed: 11/11/2022]
Abstract
The quadricuspid aortic valve (QAV) is a rare congenital anomaly that typically is unrelated to other cardiac anomalies. It usually is discovered incidentally through imaging modalities such as echocardiogram or computed tomography angiogram, during surgery, or autopsy. Some patients with QAV develop aortic regurgitation and, rarely, other cardiovascular complications like aortic aneurysm. Due to its rarity, it is difficult to characterize these patients or standardize management. However, review of case reports can be very useful in rare medical conditions such as QAV. In this manuscript, in addition to presenting two cases of QAV, the authors reviewed 149 cases of QAV from 2010 to 2020 from published case studies and series in order to gain a better insight into the characteristics of the patient population with QAV and its management.
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Affiliation(s)
- Jacqueline Sohn
- University Hospitals Cleveland Medical Center, Cleveland, OH.
| | - Faisal D Arain
- University Hospitals Cleveland Medical Center, Cleveland, OH
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10
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Shadman S, Asadi MS, Nomigolzar S, Sarfarazi M. A Congenital Quadricuspid Aortic Valve Associated With Aortic and Mitral Regurgitation: Case Report and Literature Review. Cureus 2021; 13:e12986. [PMID: 33542882 PMCID: PMC7847776 DOI: 10.7759/cureus.12986] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/25/2022] Open
Abstract
Quadricuspid aortic valve (QAV) is a rare congenital cardiac anomaly that commonly becomes symptomatic in the fifth or sixth decade of life and can present as an isolated finding or in association with other congenital cardiovascular abnormalities. Previously, QAV was mainly a postmortem or intraoperative diagnosis and data was very limited on its natural history, associated complications, and long-term outcomes. In recent decades, however, there has been an increase in the reported cases of QAV, considering the advances in the diagnostic modalities such as transthoracic echocardiography (TTE), transesophageal echocardiography (TEE), and cardiac magnetic resonance (CMR). In this article, we present a case of a congenital QAV associated with aortic regurgitation as well as briefly review the QAV classification systems, treatment options, and long-term outcomes. A 48-year-old woman with a significant past medical history (PMH) of hypertension and coronary artery disease presented with shortness of breath, chest pain, and orthopnea for two to three weeks. The patient’s TTE showed severe aortic regurgitation with significant compromise in cardiac output that could not be otherwise explained. Subsequently, her TEE revealed QAV as the underlying source of these findings. Although the diagnosis of QAV could be very challenging, it is crucial to be considered when evaluating a patient with inscrutable progressive aortic regurgitation. Today, as a result of technological advancement, QAV is being diagnosed more accurately and promptly. Since there are no universal guidelines defined for this cardiac anomaly, regular follow-up with these patients is imperative to monitor for early signs of valvular compromise and to treat accordingly through medical and surgical interventions.
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Affiliation(s)
- Shahrad Shadman
- Internal Medicine, University of Maryland Capital Region Health, Cheverly, USA
| | | | - Soroush Nomigolzar
- Clinical Foundations, Ross University School of Medicine, Bridgetown, BRB
| | - Mohammad Sarfarazi
- Cardiovascular Diseases, University of Maryland Capital Region Health, Cheverly, USA
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11
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Kariyanna PT, Francois J, Jayarangaiah A, Chowdhury YS, Grodman R, Salifu MO, McFarlane IM. Quadricuspid Aortic Valve: A Case Report and Review. AMERICAN JOURNAL OF MEDICAL CASE REPORTS 2020; 8:253-256. [PMID: 32775626 PMCID: PMC7410520] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Indexed: 11/29/2022]
Abstract
Quadricuspid aortic valve (QAV) is a rare congenital valvular abnormality with less than 200 cases reported to date. The first QAV was reported in 1862 by Balington. Clinical manifestations of patients with a QAV depend on the functional status of the QAV and the associated cardiac disorders. Most QAV are asymptomatic and are incidentally found. Severe aortic regurgitation and/or stenosis can develop overtime, requiring aortic valve replacement or repair. Transesophageal echocardiography is the preferred modality to diagnose QAV. We present a case of QAV which was incidentally diagnosed in a 43-year-old woman who presented with gastroenteritis.
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Affiliation(s)
- Pramod Theetha Kariyanna
- Department of Internal Medicine, State University of New York, Downstate Medical Center, Brooklyn NY 11203, USA
| | - Jonathan Francois
- Department of Internal Medicine, State University of New York, Downstate Medical Center, Brooklyn NY 11203, USA
| | - Amog Jayarangaiah
- Trinity School of Medicine, Ratho Mill, Ribishi, St. Vincent and Grenadines
| | - Yuvraj Singh Chowdhury
- Department of Internal Medicine, State University of New York, Downstate Medical Center, Brooklyn NY 11203, USA
| | - Richard Grodman
- Richmond University Medical Center, Department of Cardiology, Staten Island, New York, United States
| | - Moro O. Salifu
- Department of Internal Medicine, State University of New York, Downstate Medical Center, Brooklyn NY 11203, USA
| | - Isabel M. McFarlane
- Department of Internal Medicine, State University of New York, Downstate Medical Center, Brooklyn NY 11203, USA,Corresponding author:
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Tsugu T, Murata M, Endo J, Kawakami T, Tsuruta H, Itabashi Y, Fukuda K. Long-term outcomes in 3 cases of quadricuspid aortic valve, using a new classification system: A case series and literature review. Echocardiography 2019; 36:595-597. [DOI: 10.1111/echo.14278] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/10/2018] [Revised: 12/07/2018] [Accepted: 01/15/2019] [Indexed: 11/28/2022] Open
Affiliation(s)
- Toshimitsu Tsugu
- Department of Cardiology; Keio University School of Medicine; Tokyo Japan
| | - Mitsushige Murata
- Center for Preventive Medicine; Keio University School of Medicine; Tokyo Japan
| | - Jin Endo
- Department of Cardiology; Keio University School of Medicine; Tokyo Japan
| | - Takashi Kawakami
- Department of Cardiology; Keio University School of Medicine; Tokyo Japan
| | - Hikaru Tsuruta
- Department of Cardiology; Keio University School of Medicine; Tokyo Japan
| | - Yuji Itabashi
- Department of Cardiology; Keio University School of Medicine; Tokyo Japan
| | - Keiichi Fukuda
- Department of Cardiology; Keio University School of Medicine; Tokyo Japan
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13
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Correa LCS, Carlos ÉMR, Assunção PCLD, Pereira JEG, Bersot CDA. Anesthetic Management for Quadricuspid Aortic Valve Repair: Case Report and Literature Review. OPEN JOURNAL OF ANESTHESIOLOGY 2018; 08:172-182. [DOI: 10.4236/ojanes.2018.85018] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 09/18/2024]
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14
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Yuan SM. Quadricuspid Aortic Valve: A Comprehensive Review. Braz J Cardiovasc Surg 2017; 31:454-460. [PMID: 28076624 PMCID: PMC5407143 DOI: 10.5935/1678-9741.20160090] [Citation(s) in RCA: 28] [Impact Index Per Article: 3.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/09/2016] [Accepted: 07/25/2016] [Indexed: 01/19/2023] Open
Abstract
Quadricuspid aortic valve (QAV) is a rare congenital heart disease. The
functional status of QAV is predominantly a pure aortic regurgitation. Clinical
manifestations of patients with a QAV depend on the functional status of the QAV
and the associated disorders. Significant valvular regurgitation and (or)
stenosis is often present with subsequent operation performed at the fifth to
sixth decade of life. The functional status of QAV is predominantly regurgitant;
whereas pure stenotic QAV can be as few as in only 0.7% of the patients. QAV is
usually an isolated anomaly, but other congenital heart defects can be present
in 18-32% of the patients. About one-fifth of them require a surgical operation.
Tricuspidalization is a preferred technique for QAV repair. As not all the
patients with a QAV necessarily warrant a surgical operation, decision-making in
patient selection and surgical procedure of choice are crucial. Antibiotic
prophylaxis against infective endocarditis is necessary in the QAV patients with
unequal-sized cusps.
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Affiliation(s)
- Shi-Min Yuan
- The First Hospital of Putian, Teaching Hospital, Fujian Medical University, Putian, Fujian Province, China
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15
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Malviya A, Jha PK, Ashwin, Mishra J, Srivastava P, Mishra A. Quadricuspid aortic valve – A case report and literature review. Egypt Heart J 2016. [DOI: 10.1016/j.ehj.2015.09.003] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/16/2022] Open
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16
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Neurocardiogenic Syncope and Supraventricular Tachycardia in Association with a Rare Congenital Aortic Valve Abnormality. Case Rep Cardiol 2016; 2016:8136079. [PMID: 27891259 PMCID: PMC5116500 DOI: 10.1155/2016/8136079] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/14/2016] [Accepted: 10/10/2016] [Indexed: 11/17/2022] Open
Abstract
We report a case of a 26-year-old woman who presented with multiple episodes of syncope over a five-months period of time. Transthoracic echocardiogram had shown a normal functioning quadricuspid aortic valve (QAV) which was also confirmed on a transesophageal echocardiogram. Computed tomographic angiography of heart and coronary arteries showed the QAV with equal size of all aortic cusps and normal coronary arteries. Intermittent chest pain and palpitations warranted an exercise stress test. The stress test revealed normal aerobic exertion, with achievement of 101% of maximal peak heart rate. However, during peak stress, we noted a drop in her blood pressure significantly resulting in dizziness. No arrhythmias were noted during the stress test. With recurrent syncope episodes and palpitations, Holter monitoring was done, revealing supraventricular tachycardia (SVT). We discuss current available literature and coassociations with QAV. New association of QAV with SVT needs further analysis.
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17
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Song I, Park JA, Choi BH, Ko SM, Shin JK, Chee HK, Kim JS. Morphological and Functional Evaluation of Quadricuspid Aortic Valves Using Cardiac Computed Tomography. Korean J Radiol 2016; 17:463-71. [PMID: 27390538 PMCID: PMC4936169 DOI: 10.3348/kjr.2016.17.4.463] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/21/2015] [Accepted: 04/12/2016] [Indexed: 11/15/2022] Open
Abstract
Objective The aim of this study was to identify the morphological and functional characteristics of quadricuspid aortic valves (QAV) on cardiac computed tomography (CCT). Materials and Methods We retrospectively enrolled 11 patients with QAV. All patients underwent CCT and transthoracic echocardiography (TTE), and 7 patients underwent cardiovascular magnetic resonance (CMR). The presence and classification of QAV assessed by CCT was compared with that of TTE and intraoperative findings. The regurgitant orifice area (ROA) measured by CCT was compared with severity of aortic regurgitation (AR) by TTE and the regurgitant fraction (RF) by CMR. Results All of the patients had AR; 9 had pure AR, 1 had combined aortic stenosis and regurgitation, and 1 had combined subaortic stenosis and regurgitation. Two patients had a subaortic fibrotic membrane and 1 of them showed a subaortic stenosis. One QAV was misdiagnosed as tricuspid aortic valve on TTE. In accordance with the Hurwitz and Robert's classification, consensus was reached on the QAV classification between the CCT and TTE findings in 7 of 10 patients. The patients were classified as type A (n = 1), type B (n = 3), type C (n = 1), type D (n = 4), and type F (n = 2) on CCT. A very high correlation existed between ROA by CCT and RF by CMR (r = 0.99) but a good correlation existed between ROA by CCT and regurgitant severity by TTE (r = 0.62). Conclusion Cardiac computed tomography provides comprehensive anatomical and functional information about the QAV.
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Affiliation(s)
- Inyoung Song
- Department of Radiology, Konkuk University Medical Center, Konkuk University School of Medicine, Seoul 05030, Korea
| | - Jung Ah Park
- Department of Radiology, Konkuk University Medical Center, Konkuk University School of Medicine, Seoul 05030, Korea
| | - Bo Hwa Choi
- Department of Radiology, Konkuk University Medical Center, Konkuk University School of Medicine, Seoul 05030, Korea
| | - Sung Min Ko
- Department of Radiology, Konkuk University Medical Center, Konkuk University School of Medicine, Seoul 05030, Korea
| | - Je Kyoun Shin
- Department of Thoracic Surgery, Konkuk University Medical Center, Konkuk University School of Medicine, Seoul 05030, Korea
| | - Hyun Keun Chee
- Department of Thoracic Surgery, Konkuk University Medical Center, Konkuk University School of Medicine, Seoul 05030, Korea
| | - Jun Seok Kim
- Department of Thoracic Surgery, Konkuk University Medical Center, Konkuk University School of Medicine, Seoul 05030, Korea
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18
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Dagnegård HH, Røpcke DM, Lund JT. Endocarditis in a Quadricuspid Aortic Valve. Ann Thorac Surg 2016; 101:e167. [PMID: 27106469 DOI: 10.1016/j.athoracsur.2015.12.025] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/23/2015] [Revised: 12/10/2015] [Accepted: 12/14/2015] [Indexed: 10/21/2022]
Affiliation(s)
- Hanna H Dagnegård
- Department of Cardiothoracic Surgery, University of Copenhagen, Copenhagen, Denmark.
| | - Diana M Røpcke
- Department of Cardiothoracic Surgery, University of Copenhagen, Copenhagen, Denmark
| | - Jens T Lund
- Department of Cardiothoracic Surgery, University of Copenhagen, Copenhagen, Denmark
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19
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Abstract
Background—
Quadricuspid aortic valve (QAV) is a rare congenital cardiac defect. This study sought to determine QAV frequency in a large echocardiography database, to characterize associated cardiovascular abnormalities, and to describe long-term outcomes.
Methods and Results—
Fifty patients (mean±SD age, 43.5±21.8 years at the time of the index diagnosis; female sex, 52%) received a diagnosis of QAV between January 1, 1975, and March 14, 2014 (frequency, 0.006%). The QAV was type A in 32% and type B in 32% (Hurwitz and Roberts classification). Aortic dilatation was present in 29% of the patients, and 26% had moderate or severe aortic valve regurgitation at the index diagnosis. Stenosis affected only 8% of the valves and was mild. Other findings, including abnormalities of other cardiac valves, septal defects, persistent left superior vena cava, and patent ductus arteriosus, were present in 32% of patients. During a mean±SD follow-up of 4.8±5.6 years, 8 patients underwent aortic valve surgery, with severe aortic valve regurgitation being the surgical indication in 7 patients. One patient with mild to moderate aortic valve regurgitation underwent aortic valve repair for obstruction of the left coronary ostium by the accessory cusp of QAV. No infective endocarditis or aortic dissection was found. Overall survival was 91.5% and 87.7% at 5 and 10 years.
Conclusions—
Aortic dilatation and other structural cardiac abnormalities were relatively common among patients with QAV. Aortic valve regurgitation was the predominant hemodynamic abnormality and the indication for aortic valve surgery in most patients who received surgery. Long-term survival was excellent.
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20
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Yılmaz S, Yılmaz D, Demirtaş S, Keser N, Gündüz H. Rare congenital valve disease: Quadricuspid aortic valve. INTERNATIONAL JOURNAL OF THE CARDIOVASCULAR ACADEMY 2015. [DOI: 10.1016/j.ijcac.2015.08.002] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/25/2022] Open
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21
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Savino K, Quintavalle E, Ambrosio G. Quadricuspid Aortic Valve: A Case Report and Review of the Literature. J Cardiovasc Echogr 2015; 25:72-76. [PMID: 28465938 PMCID: PMC5353411 DOI: 10.4103/2211-4122.166077] [Citation(s) in RCA: 19] [Impact Index Per Article: 1.9] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/04/2022] Open
Abstract
The quadricuspid aortic valve (QAV) is a rare malformation; often isolated, sometimes associated with other heart diseases. Before the era of echocardiography, the diagnosis was made incidentally at autopsy or during surgery of valve replacement. The extensive use of echocardiography has allowed an early and accurate diagnosis of this malformation. In many cases, the transthoracic approach is suitable for the diagnosis but, transesophageal echocardiography is a tool for the accurate definition of the valve anatomy. This review analyzes, after the presentation of a clinical case, the current knowledge on embryogenesis, classification, diagnosis and clinical course of QAV.
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Affiliation(s)
- Ketty Savino
- Department of Cardiology and Cardiovascular Physiopathology, University of Perugia, Perugia, Italy
| | - Elisa Quintavalle
- Department of Cardiology and Cardiovascular Physiopathology, University of Perugia, Perugia, Italy
| | - Giuseppe Ambrosio
- Department of Cardiology and Cardiovascular Physiopathology, University of Perugia, Perugia, Italy
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22
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Massoni F, Ricci S. Death From Ascending Aortic Aneurysm Secondary to Quadricuspid Aortic Valve. Am J Forensic Med Pathol 2014; 35:232-3. [DOI: 10.1097/paf.0000000000000126] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/25/2022]
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23
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Tsujimoto S, Motohiro M, Kamihata H, Iwasaka T, Shiojima I. Quadricuspid aortic valve associated with idiopathic dilated cardiomyopathy: A case report. J Cardiol Cases 2014; 9:233-235. [DOI: 10.1016/j.jccase.2014.03.001] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/25/2013] [Revised: 02/11/2014] [Accepted: 03/05/2014] [Indexed: 10/25/2022] Open
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24
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Monigari N, Poondru RR, Kareem H, Devasia T. Quadricuspid aortic valve: a rare congenital cardiac anomaly. BMJ Case Rep 2014; 2014:bcr-2014-204162. [PMID: 24748144 DOI: 10.1136/bcr-2014-204162] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/03/2022] Open
Affiliation(s)
- Naresh Monigari
- Department of Medicine, Kasturba Medical College, Manipal, Karnataka, India
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25
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Zhu J, Zhang J, Wu S, Zhang Y, Ding F, Mei J. Congenital quadricuspid aortic valve associated with aortic insufficiency and mitral regurgitation. J Cardiothorac Surg 2013; 8:87. [PMID: 23587156 PMCID: PMC3648369 DOI: 10.1186/1749-8090-8-87] [Citation(s) in RCA: 13] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/18/2013] [Accepted: 04/09/2013] [Indexed: 01/19/2023] Open
Abstract
Congenital quadricuspid aortic valve is a rare cardiac anomaly. More than half of the patients with this abnormality will develop aortic insufficiency in adulthood. It is vital that patients with quadricuspid aortic valve who present with progressive aortic regurgitation undergo valve replacement or repair at appropriate time. Here, we present two cases of quadricuspid aortic valve. We first describe a 58-year-old man who had mitral regurgitation and ascending aorta dilatation with quadricuspid aortic valve. He underwent aortic valve replacement and mitral valve plasty and recovered well. The second patient is a 20-year-old asymptomatic boy who has been closely followed up and has not received any surgical treatment.
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Affiliation(s)
- Jiaquan Zhu
- Cardiovascular Surgery Division, Cardiac Center, Shanghai Jiaotong University, Shanghai, China
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26
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Quadricuspid aortic valve by cardiac magnetic resonance imaging: a case report and review of the literature. J Comput Assist Tomogr 2011; 35:637-41. [PMID: 21926862 DOI: 10.1097/rct.0b013e318224a129] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/26/2022]
Abstract
Quadricuspid aortic valve (QAV) is a rare congenital cardiac entity. The recognition of QAV has clinical significance as it causes aortic valve dysfunction, commonly aortic regurgitation, and is often associated with other congenital cardiac abnormalities. We showed the important role played by cardiac magnetic resonance imaging in detecting QAV and review the available literature to explain its incidence, diagnosis, classifications, embryology, correlation between morphology of the QAV and its function, associated conditions, and management.
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27
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Kuroki H, Hirooka K, Ohnuki M. Pentacuspid aortic valve causing severe aortic regurgitation. J Thorac Cardiovasc Surg 2011; 143:e11-2. [PMID: 22104681 DOI: 10.1016/j.jtcvs.2011.10.069] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/08/2011] [Accepted: 10/25/2011] [Indexed: 10/15/2022]
Affiliation(s)
- Hidehito Kuroki
- Department of Cardiovascular Surgery, Tsuchiura Kyodo General Hospital, Ibaraki, Japan.
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28
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Gouveia S, Martins JDF, Costa G, Paramés F, Freitas I, Rebelo M, Trigo C, F. Pinto F. Válvula aórtica quadricúspide – Casuística de 10 anos e revisão da literatura. Rev Port Cardiol 2011; 30:849-54. [DOI: 10.1016/j.repc.2011.09.007] [Citation(s) in RCA: 19] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/22/2010] [Accepted: 07/16/2011] [Indexed: 11/29/2022] Open
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29
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Gouveia S, Ferreira Martins JD, Costa G, Paramés F, Freitas I, Rebelo M, Trigo C, Pinto FF. Quadricuspid aortic valve – 10-year case series and literature review. REVISTA PORTUGUESA DE CARDIOLOGIA (ENGLISH EDITION) 2011. [DOI: 10.1016/j.repce.2011.10.003] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/25/2022] Open
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30
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Dynamic evaluation of a quadricuspid aortic valve with coronary computed tomography angiography. J Thorac Imaging 2011; 27:W46-7. [PMID: 21873907 DOI: 10.1097/rti.0b013e31822910d8] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/26/2022]
Abstract
A 35-year-old man presented with 9 years of chronic chest pain and was found to have moderate-to-severe aortic regurgitation on echocardiography. Aortic valve morphology on the initial echocardiogram was deemed normal. Computed tomography angiography was obtained for further evaluation of the aortic root dimension and anatomy. In addition to noninvasive evaluation of the coronary arteries, the higher spatial resolution and volumetric coverage of computed tomography angiography can better define the valvular and aortic anatomy. Reconstruction of retrospectively gated cine images through the aortic valve plane revealed a quadricuspid valve with 4 equal-sized cusps. Incomplete coaptation of the aortic valve cusps was seen during diastole, explaining the marked aortic regurgitation. In addition, a dilated ascending thoracic aorta (4.7 cm) was revealed. On the basis of these findings, the patient was treated surgically with a composite valve graft replacement of his aortic root.
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31
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Stenosed Quadricuspid Aortic Valve Treated by Transcatheter Aortic Valve Implantation. J Am Coll Cardiol 2011; 57:1567. [DOI: 10.1016/j.jacc.2010.06.065] [Citation(s) in RCA: 10] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/27/2010] [Accepted: 06/02/2010] [Indexed: 11/17/2022]
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32
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Murugesan C, Rao P, Banakal SC, Appajaiah CB, Muralidhar K, Rao PV, Shetty DP. Unusual Short-Axis Aortic Valve View. J Cardiothorac Vasc Anesth 2011; 25:199-201. [DOI: 10.1053/j.jvca.2009.12.004] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/30/2009] [Indexed: 11/11/2022]
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33
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Kane L, Puskas JD. Quadricuspid Aortic Valve Intraoperative Transesophageal Echocardiograph Image. INNOVATIONS-TECHNOLOGY AND TECHNIQUES IN CARDIOTHORACIC AND VASCULAR SURGERY 2010; 5:453-5. [DOI: 10.1177/155698451000500613] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/16/2022]
Abstract
Quadricuspid aortic valve is an extremely rare congenital anomaly. In Quadricuspid aortic valve, the aortic valve has four distinct leaflets. The most common type A variant has four equal cusps. The most common presentation is aortic insufficiency. A 70-year-old woman and a 32-year-old man presented to our practice in a 3-month time span were described here.
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Affiliation(s)
- Lauren Kane
- Division of Cardiothoracic Surgery, Emory University School of Medicine, Atlanta, GA USA
| | - John D. Puskas
- Division of Cardiothoracic Surgery, Emory University School of Medicine, Atlanta, GA USA
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34
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Xiao Z, Meng W, Zhang E. Quadricuspid aortic valve by using intraoperative transesophageal echocardiography. Cardiovasc Ultrasound 2010; 8:36. [PMID: 20813040 PMCID: PMC2939529 DOI: 10.1186/1476-7120-8-36] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/03/2010] [Accepted: 09/02/2010] [Indexed: 02/05/2023] Open
Abstract
Quadricuspid aortic valve is a rare congenital malformation of the aortic valve. Its diagnosis is often missed even with the use of transthoracic echocardiogram. Many of these patients progress to aortic incompetence later in life, hence requiring surgical intervention. In the case described in this report, a 61-year-old woman is presented with the features of congestive heart failure. The preoperative transthoracic echocardiogram disclosed a moderate to severe aortic valve insufficiency but failed to reveal the quadricuspid aortic value anomaly. This case underscores the important role of three-dimensional transesophageal echocardiography for the diagnosis of quadricuspid aortic valve.
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Affiliation(s)
- Zhenghua Xiao
- Department of Thoracic and Cardiovascular Surgery, West China Hospital, Sichuan University, ChengDu, China
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35
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Nikdoust F, Sadeghian H, Eslami B, Majidi S, Javidi D. Quadricuspid aortic valve diagnosed by transesophageal echocardiography: a case report. J Tehran Heart Cent 2010; 5:95-7. [PMID: 23074576 PMCID: PMC3466823] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/14/2008] [Accepted: 04/19/2009] [Indexed: 11/08/2022] Open
Abstract
Quadricuspid aortic valve (QAV) is an uncommon congenital anomaly which was an incidental finding during surgery or autopsy in the past. We present the case of a 44-year-old woman with moderately severe aortic regurgitation due to unequal cusp size QAV diagnosed via transesophageal echocardiography. Due to echocardiographic imaging improvement, the diagnosis of QAV is now easier and earlier than the past.
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Affiliation(s)
- Farahnaz Nikdoust
- Tehran Heart Center, Tehran University of Medical Sciences, Tehran, Iran
| | - Hakimeh Sadeghian
- Tehran Heart Center, Tehran University of Medical Sciences, Tehran, Iran
| | - Bahareh Eslami
- Tehran Heart Center, Tehran University of Medical Sciences, Tehran, Iran
| | - Shahla Majidi
- Tehran Heart Center, Tehran University of Medical Sciences, Tehran, Iran
| | - Dariush Javidi
- Shariati Hospital, Tehran University of Medical Sciences, Tehran, Iran
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36
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A case of pentacuspid pulmonary valve. Surg Radiol Anat 2009; 32:613-5. [PMID: 20024548 DOI: 10.1007/s00276-009-0607-7] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/26/2009] [Accepted: 12/01/2009] [Indexed: 10/20/2022]
Abstract
A pentacuspid pulmonary valve is a rare congenital anomaly. The present report describes a case of pentacuspid pulmonary valve encountered during the autopsy of a 50-year-old man with a ruptured abdominal aortic aneurysm. The pentacuspid pulmonary valve had three relatively equal cusps and two smaller cusps. In this case, a tricuspid aortic valve was identified with normal structure and no evidence of heart failure was found clinically or pathologically.
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37
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Abstract
OBJECTIVE The purpose of this article is to describe the spectrum of imaging findings of congenital heart disease in adults. CONCLUSION Continued advances in CT have facilitated evaluation of two important patient populations: adults with surgically palliated congenital heart disease and adults with previously undiagnosed congenital heart disease.
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38
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39
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Attaran RR, Habibzadeh MR, Baweja G, Slepian MJ. Quadricuspid aortic valve with ascending aortic aneurysm: report of a case and discussion of embryological mechanisms. Cardiovasc Pathol 2009; 18:49-52. [DOI: 10.1016/j.carpath.2007.07.012] [Citation(s) in RCA: 15] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/11/2007] [Revised: 07/12/2007] [Accepted: 07/24/2007] [Indexed: 11/26/2022] Open
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40
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Abstract
Quadricuspid aortic valves are rare congenital anomalies, usually diagnosed in adult patients affected by severe aortic regurgitation. We have now encountered three such valves in children undergoing transthoracic echocardiography. All children were asymptomatic. The first child possessed a valve with two larger leaflets, and smaller leaflets of equal size. In the second child, 3 leaflets were of equal size, with 1 small accessory leaflet. The third child had a valve with four leaflets of approximately equal size. In one child, the aortic valve was functioning normally, and came to attention because of mitral valvar prolapse causing mild regurgitation. In the remaining two children, central mild aortic regurgitation was detected using colour flow analysis, and associated dilation of the aortic root was revealed by measurements of the cross-sectional images.
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41
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Nucifora G, Badano LP, Iacono MA, Fazio G, Cinello M, Marinigh R, Fioretti PM. Congenital quadricuspid aortic valve associated with obstructive hypertrophic cardiomyopathy. J Cardiovasc Med (Hagerstown) 2008; 9:317-8. [DOI: 10.2459/jcm.0b013e328224b946] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/05/2022]
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42
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YAMAGISHI Y, YUDA S, TSUCHIHASHI K, SAITOH S, MIURA T, URA N, SHIMAMOTO K. Quadricuspid aortic valve associated with aortic stenosis and regurgitation: report of a case and a review of the literature. J Med Ultrason (2001) 2007; 34:197-200. [DOI: 10.1007/s10396-007-0148-9] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/29/2007] [Accepted: 04/20/2007] [Indexed: 10/22/2022]
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43
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Fratellone P, Berger M, Khan M, Bassiri-Tehrani M. Quadricuspid aortic valve diagnosed by echocardiography in two cases identical twins. Am J Cardiol 2007; 100:1490-1. [PMID: 17950814 DOI: 10.1016/j.amjcard.2007.06.046] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/09/2007] [Revised: 06/06/2007] [Accepted: 06/06/2007] [Indexed: 11/26/2022]
Abstract
Quadricuspid aortic valves (QAVs) are a rare but well-recognized congenital heart disease and a cause of aortic regurgitation. The first case was reported in 1862. Since then, there have been about 189 cases reported with advances in echocardiography, more cases are diagnosed by transesophageal echocardiography. Hurwitz and Roberts have classified QAV according to the size of the leaflets. Our case demonstrates 2 different valve classifications based on equal and unequal cusp size. In conclusion, to our knowledge, this is the first case of QAV in identical twins.
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44
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Croccia MG, Chiaramonti F, Pratali S, Scioti G, Guarracino F, Bortolotti U. Quadricuspid aortic valve associated with hypoplastic aortic annulus. J Thorac Cardiovasc Surg 2007; 134:1061-2. [PMID: 17903538 DOI: 10.1016/j.jtcvs.2007.07.007] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/25/2007] [Accepted: 07/05/2007] [Indexed: 11/23/2022]
Affiliation(s)
- Maria Grazia Croccia
- Section of Cardiac Surgery, Cardiac Thoracic and Vascular Department, University of Pisa Medical School, Pisa, Italy
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45
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Chang PC, Lin CC, Lee CS, Wang PH, Chen YF. Rare anatomic entity of quadricuspid aortic valve with aortic insufficiency: a case report. Kaohsiung J Med Sci 2007; 23:422-5. [PMID: 17666310 DOI: 10.1016/s0257-5655(07)70007-x] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/26/2022] Open
Abstract
Quadricuspid aortic valve (QAV) is a rare congenital heart defect that often causes symptomatic aortic insufficiency in adulthood, imposing valve replacement. Herein, we describe one unusual case of QAV which underwent valve replacement uneventfully.
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Affiliation(s)
- Po-Chih Chang
- Division of Cardiovascular Surgery, Department of Surgery, Kaohsiung Medical University Hospital, Kaohsiung, Taiwan
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46
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Holt NF, Sivarajan M, Mandapati D, Printsev Y, Elefteriades JA. Quadricuspid Aortic Valve With Aortic Insufficiency: Case Report and Review of the Literature. J Card Surg 2007; 22:235-7. [PMID: 17488428 DOI: 10.1111/j.1540-8191.2007.00399.x] [Citation(s) in RCA: 10] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/27/2022]
Abstract
We report the case of a 42-year-old woman with aortic regurgitation discovered to be caused by a quadricuspid aortic valve (QAV) diagnosed by intraoperative transesophageal echocardiogram. With improvements in echocardiographic imaging, the diagnosis of QAV is likely to be made more reliably in the future and should prompt close clinical follow-up given the frequent association of this lesion with valvular insufficiency.
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Affiliation(s)
- Natalie F Holt
- Department of Surgery, Section of Cardiothoracic Surgery, Yale University School of Medicine, PO Box 208039, New Haven, CT 06520-8039, USA
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47
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Mohamed SA, Misfeld M, Hanke T, Belge G, Bullerdiek J, Sievers HH. Quadricuspid aortic valve in a patient with Turner syndrome. Exp Clin Cardiol 2007; 12:161-162. [PMID: 18650999 PMCID: PMC2323752] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/09/2006] [Accepted: 12/04/2006] [Indexed: 05/26/2023]
Abstract
A quadricuspid aortic valve is an uncommon congenital anomaly that is often associated with other cardiac disorders. Most reported cases of quadricuspid aortic valves are detected incidentally during necropsy or aortic valve replacement and, therefore, the potential clinical course still remains unclear. A case of a 47-year-old woman with grade III to IV aortic insufficiency and mild left ventricular dilation with an end-diastolic diameter of 59 mm is presented. During surgery for aortic valve replacement (Ross procedure), a quadricuspid aortic valve was identified. Two years after the successful Ross procedure, a molecular genetic study of this rare anomaly was performed using karyotyping, fluorescence in situ hybridisation and polymerase chain reaction. Cytogenetic analysis detected chromosomal aberration 45,X0/46,XX, indicating a low-level X chromosome mosaicism; repeat karyotypes were normal. This is the first reported case of a quadricuspid aortic valve in a woman with Turner syndrome.
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Affiliation(s)
- Salah A Mohamed
- University of Schleswig-Holstein Campus Luebeck, Department of Cardiac Surgery, Luebeck
| | - Martin Misfeld
- University of Schleswig-Holstein Campus Luebeck, Department of Cardiac Surgery, Luebeck
| | - Thorsten Hanke
- University of Schleswig-Holstein Campus Luebeck, Department of Cardiac Surgery, Luebeck
| | - Gazanfer Belge
- Center for Human Genetics, University of Bremen, Bremen, Germany
| | - Joern Bullerdiek
- Center for Human Genetics, University of Bremen, Bremen, Germany
| | - Hans H Sievers
- University of Schleswig-Holstein Campus Luebeck, Department of Cardiac Surgery, Luebeck
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48
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Abstract
Quadricuspid aortic valves (QAV) are a rare but well recognized cause of significant aortic regurgitation. The first case was found reported in 1862. Since then there have been 110 reported cases of QAV and we report 4 more. Previously, these were diagnosed at the time of surgery or postmortem examination. With advances in echocardiography, including harmonic imaging, and also the advent of transesophageal echocardiography, more cases are being diagnosed prior to surgery. We describe four more cases, three diagnosed preoperatively and one at the time of surgery, and then review the previously reported cases. Of the 114 cases reported, 46 had the aortic valve replaced, most commonly in the 5th and 6th decade of life. Hurwitz and Roberts classified quadricuspid valves according to the size of the leaflets. It has previously been believed that QAVs with four equal sized leaflets were less likely to develop significant aortic regurgitation; however, on review of the available cases, this would not appear to be the case. The preoperative diagnosis of QAVs is important as they can be associated with abnormally placed coronary ostium. Of the 114 cases reported, there are 10 reports of abnormally placed ostia. There has been at least one reported case of death occurring because of obstruction of an abnormally placed right coronary ostium by a prosthetic aortic valve.
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49
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Brzezinski M, Mertz V, Clements FM, Glower DD, Puskas F, Grichnik KP, Swaminathan M. Transesophageal Echocardiography of the Quadricuspid Aortic Valve. Anesth Analg 2006; 103:1414-5. [PMID: 17122215 DOI: 10.1213/01.ane.0000243390.30837.5f] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/05/2022]
Affiliation(s)
- Marek Brzezinski
- Department of Anesthesia and Perioperative Care, VA Medical Center, University of California, San Francisco, 4150 Clement Street, San Francisco, CA 94121, USA.
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Suzuki Y, Daitoku K, Minakawa M, Fukui K, Fukuda I. Congenital quadricuspid aortic valve with tetralogy of Fallot and pulmonary atresia. ACTA ACUST UNITED AC 2006; 54:44-6. [PMID: 16482938 DOI: 10.1007/bf02743785] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/25/2022]
Abstract
We report the case of a 4-year-old girl who had quadricuspid aortic valve regurgitation with tetralogy of Fallot and pulmonary atresia. This combination is very uncommon. Aortic valve replacement was performed successfully due to aortic regurgitation which had progressed one year after the total repair. The dilated aortic annulus plus quadricuspid aortic valve may result in progressive aortic regurgitation for a short period.
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Affiliation(s)
- Yasuyuki Suzuki
- Department of Surgery I, Hirosaki University School of Medicine, Aomori, Japan
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