Ventricular and pulmonary artery volumes in patients with absent pulmonary valve. Factors affecting the natural course.

Abstract

SUMMARY Right and left ventricular (RV and LV) volumes were determined in 19 patients with absent pulmonary valve syndrome using Simpson's rule and area-length methods. The volume of the proximal right pulmonary artery (RPAV) was calculated at maximal and minimal size using the area-length method. Patient groups included four newborns who responded to medical management (group 1A), seven critically ill newborns who died (group iB), four infants ages 1-10 months (group 2) and four children ages 2-8 years (group 3). The RVend-diastolic volume in groups 1B and 2 was significantly greater than that in groups 1A and 3. The RV ejection fraction in groups lB and 2 was significantly less than that in normal patients and groups 1A and 3. Maximal RPAV correlated well with RV stroke volume and end-diastolic volume. The maximal RPAV in group 1B was significantly greater than that in groups 1A and 3. Pulmonary arterial compliance was greater than normal in all groups, and the compliance in group 1B was more than two times that in the other groups. We conclude that the increased right pulmonary artery compliance and pulmonary regurgitation in patients with absent pulmonary valve contribute to bronchial obstruction and right-heart failure and are the causes of the high morbidity and mortality in these patients. The management should be directed to the alleviation of bronchial obstruction and right-heart failure.THE ABSENT pulmonary valve syndrome (APVS) usually consists of an absent pulmonary valve, a ventricular septal defect, mild-to-severe right ventricular (RV) outflow obstruction, and aneurysmal dilatation of the pulmonary arteries. The always located at the pulmonary valve ring, and sometimes at the infundibulum as well. 1-3 This combination of the lesions is better known as tetralogy of Fallot with absent pulmonary valve.Calder et al.4 reviewed 233 cases with APVS and reported that many infants die early from severe respiratory distress and intractable cardiac failure. Bronchial compression by distended pulmonary arteries is a particular problem in patients with APVS in early infancy.2 5,6 However, the relationship between RV function and the aneurysmal dilatation of the pulmonary arteries is not known. Furthermore, it is not clear how often RV and left ventricular (LV) function influence the morbidity and mortality of these patients. In this study we describe the characteristics of RV and LV performance in newborns, infants and children

Left and Right Ventricular Function in Fetal Tetralogy of Fallot with Absent Pulmonary Valve

“…13 Predictors of Outcome in Fetal TOF/APV The second aim of our study was to identify predictive factors of outcome in fetal life. Hiraishi et al 14 previously reported that higher RV end-diastolic volume and lower RV ejection fraction angiographically in the postnatal period indicated poor outcome. Based on the findings of our study, these predictors of worse outcome may be best explained by the presence of worse RV performance.…”

Tetralogy of Fallot with Absent Pulmonary Valve: A Continuing Challenge

“…Pulmonary insufficiency secondary to absence of the valve and the left-to-right shunt across the ventricular septal defect results in volume overload of the right ventricle in addition to the increased pressure load caused by outflow obstruction, and predisposes the right ventricle to failure (10). Infants with a predominant left-to-right shunt may suffer from cyanosis and hypoxic spells.…”

“…Despite the anatomic similarity with the classic TOF, the pathophysiology is strikingly different (8,10). Patients are presented in two well-defined groups.…”