Top Things to Know: Pulmonary Hypertension in CHD
Published: June 26, 2023
- Pulmonary hypertension (PH) associated with congenital heart disease is an increasingly common cause of PH. The improvements in diagnosis and management of CHD have led to increased survival into adulthood accompanied by a significant number of associated cardiac sequelae, including PH. However, data guiding management of this form of PH are limited.
- Pulmonary hypertension associated with congenital heart disease is a heterogeneous disease that includes one or a combination of etiologies as classified by the 6th World Symposium of Pulmonary Hypertension: pulmonary arterial hypertension (Group 1, PAH), PH associated with congenital left heart disease (Group 2), PH due to pulmonary artery obstructions (Group 4) and PH associated with multifactorial mechanisms including complex congenital heart disease and univentricular heart (Group 5).
- Group 1 pulmonary arterial hypertension (PAH) associated with congenital heart disease encompasses variable physiologies with important differences in treatment strategies. These include: 1) Eisenmenger syndrome; 2) unrepaired significant shunt lesions with elevated pulmonary vascular resistance but persistent left-to-right shunting; 3) coincidental small shunt lesions; and 4) PH following repair of congenital heart disease.
- Comprehensive cardiac imaging including echocardiography and cross-sectional (CT/MR) imaging in PH-congenital heart disease includes evaluation for structural abnormalities including intra- and extra-cardiac shunt lesions, valve and vascular abnormalities, and more complex congenital heart disease, as well as thorough evaluation of hemodynamics, atrial and ventricular geometry, and function.
- Comprehensive right and left heart catheterization is required to interpret the cause and severity of PH-congenital heart disease and to tailor treatment. Baseline hemodynamics, as well as responsivity to acute vasodilator testing, can help guide operability in patients with congenital heart disease and pulmonary vascular disease on a case-by-case basis as definitive criteria for safe repair have not been established.
- Pulmonary hypertension-specific therapies including phosphodiesterase-5 inhibitors, endothelin receptor antagonists, and prostanoids should be considered in the context of specific treatment paradigms: 1) treat-to-repair approach to improve elevated pulmonary vascular resistance in hope of allowing safe complete, or partial shunt closure in patients deemed borderline for operability; 2) continued treatment of PH after repair of congenital heart disease; 3) to improve quality of life and survival in Eisenmenger syndrome (the most advanced form of PAH); and 4) to reduce pulmonary vascular resistance and improve pulmonary flow in those with univentricular circulation following staged repair.
- Pulmonary-to-systemic shunt (reversed Potts shunt) as a bridge to recovery or transplantation is an emerging therapeutic intervention with growing evidence of improved symptoms and survival in a subset of patients with coincidental or recurrent PH after repair of congenital heart disease, suprasystemic pulmonary artery pressures and adequate right ventricular function. Referral for double lung transplant with simultaneous repair of simple shunt lesions or heart-lung transplantation in select patients with complex congenital heart disease is to be considered in patients with progressive PH despite maximal medical management.
- The prognosis for PH-congenital heart disease is variable and dependent on the type of congenital heart disease and other factors. Evaluation for surgical repair must therefore take the risks associated with the late repair of congenital heart disease into account when intervention is considered.
- The relationship between healthcare disparities and PH-congenital heart disease is unknown; however, socioeconomic and geographic barriers and social determinants of health may influence access to early diagnosis and the institution of advanced therapies. When possible, these factors should be addressed to promote equitable delivery of care.
- A collaborative, multi-disciplinary approach involving cardiologists, PH subspecialty care teams, and cardiothoracic surgeons in partnership with patients and families is key to improving outcomes and quality of life for people with PH-congenital heart disease.
Citation
Jone P-N, Ivy DD, Hauck A, Karamlou T, Truong U, Coleman RD, Sandoval JP, del Cerro Marín MJ, Eghtesady P, Tillman K, Krishnan US; on behalf of the American Heart Association Congenital Cardiac Defect Committee of the Council on Lifelong Congenital Heart Disease and Heart Health in the Young; Council on Cardiopulmonary, Critical Care, Perioperative and Resuscitation; Council on Cardiovascular and Stroke Nursing; Council on Cardiovascular Surgery and Anesthesia; Council on Clinical Cardiology; and Council on Peripheral Vascular Disease. Pulmonary hypertension in congenital heart disease: a scientific statement from the American HeartAssociation [June 26, 2023]. Circ Heart Fail. doi: 10.1161/HHF.0000000000000080