Some patients undergoing cardiopulmonary surgery to correct congenital heart defects may develop pulmonary arterial hypertension (PAH) after the immediate postoperative period.


Risk factors:

(1) timing the shunt correction too late

(2) failing to surgically correct the defect

(3) long-standing elevation of the right ventricular afterload


Differential diagnosis:

(1) pulmonary venous hypertension

(2) constrictive pericardium

(3) ventilatory lung disease

(4) thromboembolic lung disease

(5) portal hypertension

(6) drug-induced


Management involves:

(1) aggressive medical management of immediate problems

(2) surgery to relieve pericardial constriction if present

(3) anticoagulation if thromboembolic disease present

(4) relief of portal hypertension if present

(5) pharmaceutical pulmonary artery vasodilation with phosphodiesterase inhibitors, prostacyclins and/or endothelin receptor antagonit


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