PDA stenting in 6-month-old infant with suprasystemic pulmonary hypertension as a treatment option for hypertensive crisis

摘要

Introduction Pulmonary arterial hypertension is a severe disease with limited therapeutic options. When pharmacological treatment for children under 1 year is insufficient, surgical palliative options include the Potts shunt, creating an interatrial connection and interventional PDA stenting. Aim We present a case of a 6-month-old patient with suprasystemic pulmonary hypertension in whom we implanted a stent into the residual persistent arterial duct allowing decompression of the right ventricle and clinical stabilization. Case report A 6-month-old patient weighing 5.5 kg was admitted to the Pediatric Cardiology Department for cardiac catheterization due to pulmonary hypertension (PH). PH was diagnosed at 2 months and oral medication was started. Regular outpatient monitoring showed failure to thrive, progression of echocardiographic changes with growing disproportion between ventricles due to right ventricle dilatation, and rising NTproBNP levels (4214 pg/ml to 8111 pg/ml before intervention). Echocardiographic examination upon admission showed an enlarged right ventricle with hyperkinetic interventricular septum, residual 0.5–1 mm patent ductus arteriosus (PDA) shunting right-left with pressure gradient 55 mm Hg, small foramen ovale with no visible shunting across the interatrial septum, dilated right ventricle, borderline diameter of the mitral valve, aortic valve and compressed left ventricle (Figure 1). During hospitalization due to rapid deterioration of the patient’s condition, poor response to oral medication (maximum dose of sildenafil), and progressing signs of low cardiac output we decided on urgent cardiac catheterization with implantation of a stent into the patent ductus arteriosus to minimize the risk of pulmonary hypertensive crisis. We prepared an appropriate anesthetic management plan and were ready to treat pulmonary hypertensive crisis if it occurred during intervention. After initiation of general anesthesia cardiac catheterization was performed through the femoral vein and artery, and pressure measurements were taken in the right atrium, right ventricle and pulmonary artery. Pulmonary wedge pressure was assessed. The catheter was then threaded through the small patent foramen ovale (PFO) allowing the measurement of pressures in the left atrium and ventricle. Taking into account all measurements pulmonary hypertension was confirmed (Figure 2). After passing coronary guidewire 0.014...