Postnatal Hypertrophy and Dysfunction of the Right Ventricle with Cyanosis as Sequelae of Missed Diagnosis of Prenatal Duct Closure: Presentation of 5 Cases and Review of the Literature

Introduction: The prevalence of prenatal duct-closure is unknown and probably underestimated. Depending on the time of fetal closure this condition causes a wide range of postnatal clinical pathology from mild cyanosis and diastolic dysfunction of the right heart to respiratory insufficiency in severe right heart disease. Differential diagnoses for the postnatal findings include cyanotic congenital cardiac defects, persistent pulmonary hypertension, right ventricular cardiomyopathy and normally easier to distinguish Chiari network or Eustachian valve. Without prenatal suspicion establishing the postnatal diagnosis of premature duct closure is often difficult and considered only retrospectively from the clinical course.

Methods: In our retrospective single-center study, neonatal cardiac database was searched from 2010 to 2014. We excluded all patients with isolated arrhythmia, confirmed congenital heart disease or an open arterial duct. The remaining patients were reviewed to identify patients where ductal dysfunction may have been the cause for pathology.

Results: We present fivepatients with unremarkable pregnancy with different levels of postnatal diastolic right heart hypertrophy and respiratory distress, in whom clinical findings and clinical course strongly suggest the diagnosis of premature duct closure. Therapeutic interventions ranged from merely oxygen supplement to propranolol and surgery.

Conclusion: The reported cases demonstrate that many cases are not identified prenatally and the postnatal diagnosis might also be missed quite frequently. Increased awareness of this pathology could help to better define the prevalence and understand the hemodynamic impact of prenatal duct closure.