Key result
Perioperative PAH drugs facilitate successful complex CHD repair, reducing mPAP by ~79%.
Why the study?
It is unclear which adult patients with congenital heart defects previously considered inoperable due to severe pulmonary arterial hypertension are appropriate for treatment and how they should be managed with newer PAH-specific drugs.
Does surgical repair combined with PAH-specific drugs improve hemodynamics and symptoms in an adult patient with complex congenital heart disease and severe PAH?
Case Report (n=1)
No
Does surgical repair combined with PAH-specific drugs improve hemodynamics and symptoms in an adult patient with complex congenital heart disease and severe PAH?
Absolute Event Rate: 22% vs 105%
Perioperative and continuous use of PAH-specific drugs enabled successful surgical repair of complex congenital heart disease in an adult patient with severe PAH, significantly improving hemodynamics and exercise capacity.
Hypothesis-generating for perioperative PAH therapy enabling repair in severe PAH-CHD; leaves open need for controlled studies before practice change.
Background Some patients with congenital heart defects are considered inoperable because of severe pulmonary arterial hypertension (PAH) at birth, and some of these patients are followed for a long period. Recently, PAH-specific drugs have been developed to treat PAH. These drugs might make it possible to operate in adult patients with congenital heart defects who were previously considered inoperable. However, it is unclear which cases are appropriate for treatment and how they should be managed. Case presentation We describe a 32-year-old woman who was diagnosed with a ventricular septal defect, patent ductus arteriosus, and coarctation of the aorta, as well as severe PAH at birth. The attending physician at the time decided not to perform total repair. We re-evaluated the patient’s haemodynamics at 32 years of age. Her mean pulmonary artery pressure was 105 mmHg, pulmonary vascular resistance was 14.5 Wood units and pulmonary blood flow-to-systemic blood flow ratio (Qp/Qs) was 2.8. However, she had good vasoreactivity to oxygen. Vasoreactivity testing with 100% oxygen showed decreases in the following measures: the mean pulmonary artery pressure was 88 mmHg, pulmonary vascular resistance was 3.8 Wood units, and Qp/Qs was 10.8. We repaired the cases of coarctation of the aorta, patent ductus arteriosus and ventricular septal defect without the occurrence of a pulmonary hypertension crisis after surgery by using PAH-specific drugs. Furthermore, the continuous use of PAH-specific drugs improved the patient’s symptoms and the 6-min walk distance and decreased the mean pulmonary artery pressure and pulmonary vascular resistance. Her mean pulmonary artery pressure was 22 mmHg, and her pulmonary vascular resistance was 4.7 Wood units at the one-year follow-up. Conclusions With perioperative use of PAH-specific drugs, CHDs can be repaired without the occurrence of a pulmonary hypertension crisis after surgery in patients with a good vasoreactivity response to oxygen, even if the PVR is high. Medical treatment and surgery can therefore treat PAH and improve patients’ exercise capacity over time.
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Akagi et al. (2020) conducted a case report in Complex congenital heart disease with severe pulmonary arterial hypertension (n=1). PAH-specific drugs and surgical repair vs. Baseline was evaluated on Mean pulmonary artery pressure (mmHg). Perioperative use of PAH-specific drugs enabled successful surgical repair of complex congenital heart disease in a patient with severe PAH, reducing mean pulmonary artery pressure from 105 to 22 mmHg.
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