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Echocardiographic determinants of clinical course in infants with critical and severe pulmonary valve stenosis
J P Kovalchin1, T J Forbes, M R Nihill
1Lillie Frank Abercrombie Section of Pediatric Cardiology, Texas Children's Hospital, Baylor College of Medicine, Houston, USA.
Insights
Pulmonary stenosis (PS) patients showed significant right heart growth after balloon valvuloplasty. Critical PS infants had smaller tricuspid valve diameters and right ventricular volumes, potentially explaining cyanosis.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Disease
- Echocardiography
Background:
- Pulmonary stenosis (PS) is a critical congenital heart defect.
- Growth patterns of right heart structures post-pulmonary valvuloplasty are not well-defined.
- Factors influencing cyanosis in infants with PS require further investigation.
Purpose of the Study:
- To assess pulmonary valve (PV) annulus and right heart growth after balloon dilation in critical and severe PS.
- To identify morphometric and hemodynamic differences between cyanotic (critical PS) and asymptomatic (severe PS) infants.
- To correlate these differences with clinical presentations.
Main Methods:
- Echocardiography to measure PV annulus, tricuspid valve (TV) annulus, and pulmonary arteries.
- Calculation of Z values and right ventricular (RV) volumes from angiograms.
- Review of hemodynamic data and balloon pulmonary valvuloplasty techniques.
Main Results:
- Balloon pulmonary valvuloplasty was successful in 64% of critical PS and 90% of severe PS patients.
- Pulmonary valve, TV, and pulmonary arteries grew post-valvuloplasty, paralleling or exceeding somatic growth.
- Critical PS infants had smaller initial TV diameter and RV volumes compared to severe PS infants.
Conclusions:
- Right heart structures demonstrate significant growth post-balloon pulmonary valvuloplasty in infants with PS.
- Smaller TV diameter and RV volume in critical PS infants may predispose to increased right-to-left atrial shunting.
- These morphometric differences likely contribute to varied clinical presentations in infants with PS.
Objectives:
The purposes of this study were to determine the growth pattern of the pulmonary valve (PV) annulus and right heart structures in patients with critical and severe pulmonary stenosis (PS) after balloon dilation, and to determine any morphometric or hemodynamic differences between cyanotic infants with critical PS and asymptomatic infants with severe PS that may account for their varied clinical presentations.
Background:
Growth of the PV annulus and right heart structures in patients with critical PS after balloon valvuloplasty has not clearly been defined. In addition, the anatomic and hemodynamic factors that determine whether an infant with severe PS will present with cyanosis or without symptoms are not well understood.
Methods:
Measurements of the PV annulus, tricuspid valve (TV) annulus and main, right and left pulmonary arteries were obtained from initial and follow-up echocardiograms, and Z values were calculated. Hemodynamic data and balloon pulmonary valvuloplasty techniques were reviewed. Right ventricular (RV) volumes were measured from angiograms.
Results:
Fourteen patients with critical PS (mean [+/- SD] age 0.21 +/- 0.37 months) and 20 patients with severe PS (mean age 2.6 +/- 2.9 months) were evaluated at presentation and at 32 +/- 33 and 42 +/- 32 months of follow-up, respectively. Balloon pulmonary valvuloplasty was successful in 64% of patients with critical PS and in 90% of patients with severe PS. The PV, TV and pulmonary arteries increased in size after balloon pulmonary valvuloplasty in both groups at a rate that paralleled or exceeded the rate of somatic growth. The initial TV diameter and RV volume were smaller in patients with critical PS than in those with severe PS (p < 0.05 and p < 0.0008, respectively).
Conclusions:
After balloon pulmonary valvuloplasty in infants with critical and severe PS, right heart structures increase in size at a rate that parallels or exceeds the rate of somatic growth. The primary morphometric differences between these groups are a smaller TV diameter and RV volume in infants with critical PS. This may contribute to increased right to left atrial shunting and account for the variations in clinical presentation.