Diagnostic pitfalls: unexpectedly severe pulmonary hypertension with pulmonary hemorrhage after atrial septal defect closure - a case report

Abstract

Introduction Certain elderly patients may suffer from an uncorrected congenital heart disease (CHD) and severe pulmonary hypertension (PHT). Methods A 69-year-old male patient presented for elective surgical closure of an atrial septal defect (ASD) and for a radiofrequency (RF) ablation of the pulmonary veins due to frequent episodes of atrial fibrillation. A transthoracic echocardiography revealed a small defect in the middle part of the atrial septum with a pulmonary to systemic blood flow ratio of 3:1 and a right ventricle (RV) systolic pressure of 45 mmHg. Results Following the cardiopulmonary bypass institution, surgical inspection detected a sinus venosus defect with anomalous drainage of the right upper pulmonary vein into the left atrium, apart from the ASD type secundum. A pericardial patch reconstruction and Cox-Maze IV procedure was performed. Several hours after the intervention, blood was noted, refluxing from the endotracheal tube. Simultaneously, a severe hemodynamic instability appeared with nodal cardiac rhythm, due to a malposition of the atrial pace-maker wire. Pulmonary artery pressure measured 45/35 mmHg, pulmonary capillary wedge pressure (PCWP): 16 mmHg, right heart chambers were enlarged and RV contractility was severely reduced. A chest X-ray and a CT scan revealed massive hemorrhage in the right upper pulmonary lobe. A bronchial blocker was placed immediately and a resection of the affected lobe was performed. Discussion This case illustrates underestimated severity of pulmonary hypertension under conditions of the reduced RV function. An assessment of PCWP indicates precapillary PHT. The permanent left to right shunt with increased pulmonary blood flow resulted in progressive endothelial and medial disturbances of the pulmonary vessels, and in creation of the complex vascular lesions with consequential intrapulmonary hemorrhage. Adult patients with CHD remain a high-risk perioperative population, requiring all available resources and a multidisciplinary approach.