[Critical pulmonary stenosis in infants (author's transl)]

Area of Science:

• Pediatric Cardiology
• Congenital Heart Defects

Background:

• Pulmonary stenosis with a complete interventricular septum is a severe congenital heart defect.
• Clinical presentation often includes cyanosis, congestive heart failure, and hypoxic crises.

Purpose of the Study:

• To describe the clinical and diagnostic features of pulmonary stenosis with a complete interventricular septum in infants.
• To evaluate methods for assessing stenosis severity and discuss associated mortality.

Main Methods:

• Clinical data from eighteen infants with pulmonary stenosis and a complete interventricular septum were analyzed.
• Diagnostic challenges and the utility of various parameters for evaluating stenosis severity were discussed.

Main Results:

• Frequent symptoms included precocious cyanosis, congestive cardiac insufficiency, and hypoxic crises in 25% of cases.
• Clinical diagnosis was supported by cardiomegaly of right cavities and pulmonary ischemia, suggesting severe infundibular obstruction.
• Significant differences were noted among parameters used to evaluate stenosis severity.

Conclusions:

• The concept of a "right diminutive ventricle" was considered, associated with an rS electrocardiographic pattern in V1.
• High spontaneous and postsurgical mortality rates are characteristic of this condition, consistent with published series.