Open AccessTetralogy of Fallot with Absent Pulmonary Valve Syndrome with Absent Left Pulmonary Artery - A Rare Presentation
Author(s) -
Abul Kalam Shamsuddin,
Prodip Kumar Biswas,
Muhammad Ishtiaque Sayeed Al Manzoo,
Md. Abul Kalam Azad,
Md. Faruk Hasan,
Jasmin Hosain,
Mohammad Sharifuzzaman Shamsuddin
Publication year - 2020
Publication title -
bangladesh heart journal
Language(s) - English
Resource type - Journals
eISSN - 2521-3113
pISSN - 1024-8714
DOI - 10.3329/bhj.v35i1.49148
Subject(s) - tetralogy of fallot , medicine , cardiology , pulmonary artery , pulmonary valve , right pulmonary artery , presentation (obstetrics) , heart defect , girl , left pulmonary artery , surgery , heart disease , psychology , developmental psychology
Absent left pulmonary artery with Tetralogy of Fallot (TOF) with absent pulmonary valve syndrome (APVS), is a rare congenital cardiac anomaly. Here we present such a case of A 2 year 11 month old girl with cyanosis, exertional dyspnoea. Her diagnosis is confirmed by echocardiography and CT angiogram. There are very few cases have been reported till date with high postoperative mortality. Although per operative decision making was challenging regarding pulmonary valve and size of the RPA, we performed ICR with RPA reductionplasty and creation of monocuspid pulmonary valve with success. As it is a rare association and we have overcome the hindrance we came across per operatively, we are reporting this case.
Bangladesh Heart Journal 2020; 35(1) : 74-77