Tetralogy of Fallot with absent pulmonary valve syndrome: A 34-year African single centre experience

Mammen, Vijay; Adams, Paul; Ntsinjana, Hopewell; Cilliers, Antoinette

doi:10.24170/15-2-3046

Cited by 3 publications

(2 citation statements)

References 10 publications

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“…1 of 7 cases with pulmonary atresia, VSD, and major aortopulmonary collateral artery had QAV [ 82 ]. 1 of 15 cases with tetralogy of Fallot and absent pulmonary valve was associated with QAV [ 83 ].…”

Section: Discussionmentioning

confidence: 99%

Quadricuspid Pulmonary Valve: Case Report and the Comparison with Quadricuspid Aortic Valve

Miyake

Inoue

Mushiake

2023

CCR

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Background: Quadricuspid pulmonary valve (QPV) is a rare congenital anomaly. Simple QPV had been mainly diagnosed at the time of autopsy before 2000 and the frequency rates of QPV are approximately 0.02%–0.41%. QPV was initially diagnosed using transthoracic echocardiography (TTE) after 2000 and with contrast computed tomography (CT) or cardiac magnetic resonance imaging (CMR) after 2009. Obtaining the cross-sectional view of the pulmonary valve using TTE is difficult. We aimed to review the papers regarding the incidence, embryology, diagnosis, associated congenital heart anomalies, and prognosis in patients with QPV, and furthermore to compare with those in patients with quadricuspid aortic valve (QAV). Case presentation: We diagnosed QPV with mild stenosis in a 12-month-old infant. With a slight angulation of the transducer superiorly from the left high parasternal short-axis view, a short-axis view of QPV was obtained. Results: In QPV cases diagnosed at autopsy, Hurwitz’s type-b with three equal cusps and one smaller cusp is dominant, whereas Hurwitz’s type-a with four equal cusps is dominant in clinically diagnosed cases. Patients with associated congenital heart anomaly and valvular stenosis are more frequent in patients with QPV than in patients with QAV. Coronary artery anomalies and infectious endocarditis are more frequent in patients with QAV than in patients with QPV. The incidence of PR is more common in type-a QPV than in type-b QPV. There is no difference between type-a QAV and type-b QAV with respect to the incidence of aortic regurgitation (AR). It is assumed that QPV is a risk factor for a Ross operation. However, QPVs have been used as autografts in certain patients. Conclusion: Between QPV and QAV, various differences were found about frequency rates, diagnostic methods, valve morphology, valve function, associated congenital heart diseases, and frequencies of infectious endocarditis.

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Section: Discussionmentioning

confidence: 99%

Quadricuspid Pulmonary Valve: Case Report and the Comparison with Quadricuspid Aortic Valve

Miyake

Inoue

Mushiake

2023

CCR

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“…For those rare patients who survive beyond infancy, elective repair can be done before the onset of hemodynamic deterioration or respiratory complications associated with the progressive dilatation of the central pulmonary arterial system [3]. There is currently no literature emanating from Africa that has been published on Tetralogy of Fallot with absent pulmonary valve syndrome [4]. Therefore, we present a case of Tetralogy of Fallot with pulmonary valve agenesis in an adult who underwent surgical repair in a Sub African center.…”

Section: Introductionmentioning

confidence: 99%

Surgical Treatment of Tetralogy of Fallot with Pulmonary Valve Agenesis in a 22 Years Old Patient

Ba¹,

Yannick²,

Diop³

et al. 2021

WJCS

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Agenesis of pulmonary valve is a rare variant and severe form of Tetralogy of Fallot (ToF). The evolution is usually marked by respiratory and cardiac failure at early age, which needs early surgical correction. Uncorrected treatment of Tetralogy of Fallot diagnosed at adult age is infrequent and only few studies have been described. We present here a rare case of a 22 years old patient who presented with dyspnea since childhood. Subsequent investigations allowed diagnosis of treatment of Tetralogy of Fallot with agenesis of the pulmonary valve. Following the assessment, the patient underwent a surgical repair and the recovery was uneventful. The management of treatment of Tetralogy of Fallot with pulmonary valve agenesis in adult period remains complex, requiring different surgical techniques.

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Tetralogy of Fallot with Absent Pulmonary Valve: A Single-center Experience

Majumder,

Chakraborty,

Roy

et al. 2024

International Journal of Advanced Medical and Health Research

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Tetralogy of Fallot (TOF) with absent pulmonary valve (APV) is a rare congenital heart disease with a 2% incidence among patients of TOF. The main hallmark of the disease is the presence of a rudimentary pulmonary valve or a ridge-like structure with pulmonary regurgitation and a hugely dilated main pulmonary artery. It has a perinatal mortality of 14%–64%, depending on the severity of the disease. Surgical correction is done urgently in severely symptomatic patients, whereas surgery can be delayed in asymptomatic or mildly symptomatic patients. In the last 2 years (July 2021–June 2023) in our single tertiary care center, five patients of TOF with APV with varying severity have been documented. Among these five patients, two patients had cyanosis and three patients had recurrent cough and cold during childhood. Two patients were diagnosed during health screening. One newborn had respiratory distress due to compression of the tracheobronchial tree by a hugely dilated pulmonary artery. TOF with APV has features of both pulmonary stenosis and regurgitation, a malaligned ventricular septal defect, and a dilated pulmonary artery. The direction of the shunt depends on the severity of pulmonary stenosis. Patients with left-to-right shunt may develop pulmonary artery hypertension depending on flow and gradient across the pulmonary valve. Patients with a massively dilated pulmonary trunk obstructing the tracheobronchial tree and patients with right-to-left shunt and cyanosis usually have worse outcomes. Often, the disease is diagnosed in late childhood, particularly in mildly symptomatic and asymptomatic patients.

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Tetralogy of Fallot with absent pulmonary valve syndrome: A 34-year African single centre experience

Cited by 3 publications

References 10 publications

Quadricuspid Pulmonary Valve: Case Report and the Comparison with Quadricuspid Aortic Valve

Quadricuspid Pulmonary Valve: Case Report and the Comparison with Quadricuspid Aortic Valve

Surgical Treatment of Tetralogy of Fallot with Pulmonary Valve Agenesis in a 22 Years Old Patient

Tetralogy of Fallot with Absent Pulmonary Valve: A Single-center Experience

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