Aorto Pulmonary Window in Infant

Amar Taksande; V Gouthami; Sumanta Padhi; Kinjal Bakshi

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Amar Taksande, V Gouthami, Sumanta Padhi, Kinjal Bakshi.

Department of Pediatric Cardiology, Innova Children Heart Hospital And Research Centre, Hyderabad, India.

ADDRESS FOR CORRESPONDENCE
Dr. Amar Taksande, Innova Children Heart Hospital And Research Centre, White House, Tarnaka, Hyderabad, A.P. 500017, India.
Email: amar_bharti2000@yahoo.co.uk

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Keywords

Aortopulmonary window, Congenital heart disease, Infant

A 3 month old male infant, weighing 4.6kg was referred for the assessment of a heart murmur. History of breathlessness, noisy breathing since last 2 months and not feeding well since last 3 days was present. He was a full-term delivery born by emergency caesarean section for non progression of labour. On physical examination, heart rate was 130/min, respiratory rate was 48/min, blood pressure was 106/62 mmHg and oxygen saturation was 92%. There was no respiratory distress. All the peripheral pulses were well felt. On auscultation, first and second heart sounds were present with P2 louder than A2. Third heart sound was present. Ejection systolic murmur was best heard in the upper left parasternal area. On investigation, complete blood cell count showed hemoglobin of 9.3gm%, hematocrit of 30%, white cell count of 10,000/cumm, and platelet count of 3.8lakhs/cumm. Chest X-ray revealed an enlarged cardiac silhouette (CT>0.55) and increased pulmonary vasculature. Electrocardiography (EKG) showed sinus rhythm, normal QRS axis with biventricular hypertrophy. Echocardiography (fig1) showed large (13mm) distal aorto-pulmonary window involving the right pulmonary artery origin with bidirectional shunt with hyperkinetic pulmonary hypertension. There was mild tricuspid regurgitation with moderate pulmonary regurgitation (gradient=56mmHg). Left atrium and left ventricle was dilated. Subsequently, surgical closure of AP window was done. Post operative echo revealed no residual flow, normal left pulmonary artery & right pulmonary artery with good biventricular function. On follow-up after two month, child is doing well.

Figure 1: Echocardiography shows the large aorto-pulmonary window.

Figure 1: Echocardiography shows the large aorto-pulmonary window.

An Aortopulmonary window (APW) is usually wide, causing important symptoms in the first weeks or months of life, which result from the significant left-to-right shunt (1,2). In 1936, Abbot reported that the APW is a rare congenital cardiac anomaly accounting for about 0.1% of all cardiac defects in an autopsy study. This anomaly exists causing a communication between the ascending aorta (AA) and the pulmonary artery (PA) with normal separation of the aortic and pulmonary valves, which resulted from faulty embryogenesis of septation of the aortopulmonary trunk. John Elliotson first described it in 1830 and Gross performed the first successful surgery in 1948 (3,4). APW is classified into three types according to proximal, distal or total defects as per Mori's classification (5,6). Hemodynamic abnormalities of AP window mimic a large Ventricular Septal Defect (VSD) or Patent Ductus Arteriosus (PDA) with pulmonary hypertension. The heart murmur of this anomaly is often mistaken for the murmur of a high VSD, because although a continuous murmur may be present in patients with APW, more often there is only a systolic murmur that is generally heard along the upper left sternal border. Congestive heart failure (CHF) and pulmonary hypertension appear usually during early infancy. The clinical diagnosis of APW, although difficult to make, should be considered in any patient with congestive heart failure and low weight gain. EKG and chest X-ray are nonspecific, the echocardiogram is very important for diagnosing the APW (7,8). Garver et al. found that noninvasive imaging with echocardiography and/or MRI adequately defined the anatomical defects before surgery (9). Transcatheter closure of APW should be considered when anatomy is favorable in terms of location and size of the defect, in the absence of associated anomalies. In our case, the aorto pulmonary window was distal and large size, which was not possible to close by device. Hence, surgical closure of the APW was done. The Rashkind double umbrella device, the Amplatzer duct occluder, the Amplatzer septal occluder, muscular VSD occluder, and perimembranous VSD occluder have all been used to close small (type I) defects (10-12). Surgery is indicated as soon as the diagnosis is established, regardless of the patient's age when Eisemenger syndrome does not exist. The choice of the surgical technique is based upon the type and size of the AP window. The aorta and pulmonary artery may be divided, and the defects in the walls may be closed primarily or with patch material. Alternatively, the aorta or pulmonary artery may be opened and the defect patched using autologous, homologous, xenograft, or synthetic material. The prognosis of the patient with APW is excellent if surgical correction is performed early in life, before irreversible pulmonary vascular changes occur.

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Funding None

Conflict of Interest None

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