Introduction: Pulmonary atresia with intact ventricular septum (PAIVS) is congenital heart disease (CHD) with patent ductus arteriosus and the complete obstruction of the right ventricular outflow tract as a result of pulmonary valve atresia. It occurs in 4 to 8 per 100,000 live births, representing 1 to 3% of all heart defects in children.

Case report: A 17-year-old primigravida without previous prenatal controls and her preterm newborn 30 weeks of gestation is admitted to the hospital. The infant showed signs of central cyanosis, with persistent hypoxia and metabolic acidosis; echocardiogram showed the absence of the right ventricular outflow tract (tricuspid atresia) with an intact interventricular septum, confirming the diagnosis of PA-IVS. Her evolution was slow, and she showed signs of low systemic blood flow, ultimately resulting in death.

Conclusions: PA-IVS is a rare and complex CHD with a broad spectrum of presentation. Echocardiographic imaging plays a vital role in the diagnosis of this disease. An interprofessional approach, along with clinical and imaging knowledge of this disease is essential to provide the appropriate monitoring, early guidance, and effective treatment that improves the survival rate of this anomaly.