Intrapulmonary artery septation for unilateral absence of the pulmonary artery

We describe a seven-month-old boy with tetralogy of Fallot and an absent left pulmonary artery. Due to the diminutive size of the left pulmonary artery, we performed a native tissue left pulmonary artery reconstruction and intrapulmonary artery septation procedure with a left modified Blalock-Taussig shunt. After confirming left pulmonary artery growth, the patient underwent tetralogy of Fallot repair, removal of septation patch, and division of the Blalock-Taussig shunt. Nine months post-surgery, we confirmed his balanced lung perfusion (R/L ratio 6:4). The intrapulmonary artery septation procedure would be suitable for both the resuscitation and reconstruction of the hypoplastic absent pulmonary artery.

Coronary artery to pulmonary artery collateral as the principal source of pulmonary blood supply in pulmonary atresia and ventricular septal defect with absent left pulmonary artery

Pulmonary atresia and ventricular septal defect is associated with variable sources of pulmonary blood supply. We present a case of a coronary artery to pulmonary artery collateral as the principal source of pulmonary blood supply.

Tetralogy of Fallot with Absent Pulmonary Valve Syndrome with Absent Left Pulmonary Artery - A Rare Presentation

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

Reanastomosis and rehabilitation of interrupted left pulmonary artery derived from atretic ductus

True absence of a branch pulmonary artery is rare. We identified a patient initially diagnosed with an absent left pulmonary artery at a previous hospital. Due to disagreement in the initial diagnosis, she had a diagnostic catheterisation, which revealed an isolated left pulmonary artery off the left innominate artery via a ductus. The ductus was recanalised with serial stenting and balloon dilatation followed by reanastomosis to the main pulmonary artery. In a patient who initially is diagnosed with an absent pulmonary artery, an alternative diagnosis, such as this case report, should be considered.

Unrepaired Tetralogy of Fallot with Absent Pulmonary Valve in a Mildly Symptomatic 16-Year-Old Boy

Absent pulmonary valve is a rare and severe variant seen in only 3% to 6% of patients with tetralogy of Fallot. Fetuses with this combined condition who survive through birth typically need intervention in infancy or early childhood because of respiratory distress, heart failure, or failure to thrive. We describe the unusual case of a mildly symptomatic 16-year-old boy with these conditions who underwent successful primary repair. Our search of the medical literature yielded fewer than 5 cases of tetralogy of Fallot with absent pulmonary valve (or variants with an absent left pulmonary artery) and survival without repair into later adolescence or adulthood.