Archives of the Turkish Society of Cardiology
Absent Pulmonary Valve Syndrome: Analysis of 15 Patients [Turk Kardiyol Dern Ars]
Turk Kardiyol Dern Ars. 1997; 25(2): 93-100

Absent Pulmonary Valve Syndrome: Analysis of 15 Patients

Gül Sağın SAYLAM1, Ayşe SARIOĞLU1, Resmiye BEŞİKÇİ1, Gülhis BATMAZ1, Y.Barbaros KINOĞLU1, Tayyar SARIOĞLU1

Absent pulmonary valve syndrome (APVS) is a rare congenital cardiac malformation characterized by a rudimentary or dysplastic pulmonary valve, ancurysmal dilatation of the main and proximal branch pulmonary arteries. This study reports our experience with 15 patients with absent pulmonary valve syndrome seen in our department between 1988 and 1995. 4 girls and 11 boys aged 2 days-17 years (mean 2.5±5.0 years, median 6 days) were retrospectively analyzed regarding their symptoms, physical findings, ECG, chest x-ray, echocardiographic features, clinical course and management. 2 patients had isolared APVS and 13 patients had Fallot's tetralogy with absent pulmonary valve (TOF-APVS). Diagnosis was made by two-dimensional and colour flow Doppler echocardiography in each case; preoperative cardiac catheterisation and angiography was performed in 3 patients. The follow-up period in 14 patients ranged from 2 months to 7.5 years (mean 2.1±2.1 years), one patient was lost to follow-up. In 12 patients with TOF-ARVS, cyanosis and respiratory symptoms due to infection and bronchial compression by the massively dilated pulmonary arteries developed in early infancy. In 2 of these patients, spontaneous relief of symptoms was noted after 18 months of age. 3 patients, two of whom had isolated APVS, remained free of respiratory symptoms until Iate childhood. 5 patients with TOF-APVS underwent a total correction operation at the age of 2 months- 17 years. Right ventricular outflow tract reconstruction using a transannular patch was performed in 2 patients, and a hand-made monocusp perkardial pulmonary valve was inserted in one; these 3 patients are clinically well 1-4.5 years after the operation. In 2 patients with anomalous origin of the left anterior descending coronary artery from the right coronary artery, an extracardiac valveless conduit was placed between the right ventricle and the pulonary artery; both died in the early postoperative period. Our current approach to the management of APVS is to give intensive medical therapy to symptomatic infants, and to delay surgical correction for at least until 18 months, since spontaneous relief of symptoms may occur during this period. Those who survive infancy should undergo elective repair preferably with the in sertion of a pulmonary valve. Early surgical intervention should be considered in infants who fail to respond to medical management.

How to cite this article
Gül Sağın SAYLAM, Ayşe SARIOĞLU, Resmiye BEŞİKÇİ, Gülhis BATMAZ, Y.Barbaros KINOĞLU, Tayyar SARIOĞLU. Absent Pulmonary Valve Syndrome: Analysis of 15 Patients. Turk Kardiyol Dern Ars. 1997; 25(2): 93-100
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