Elsevier

Cardiology Clinics

Volume 33, Issue 4, November 2015, Pages 599-609
Cardiology Clinics

Pulmonary Hypertension in Congenital Heart Disease: Beyond Eisenmenger Syndrome

https://doi.org/10.1016/j.ccl.2015.07.003Get rights and content

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Key points

  • Pulmonary hypertension is present in approximately 5% of patients with adult congenital heart disease. These patients have worse functional status and increased mortality.

  • There are various causes of pulmonary hypertension in patients with congenital heart disease. These causes include increased pulmonary blood flow, pulmonary vascular remodeling, and pulmonary venous hypertension. There is considerable overlap in patients with congenital heart disease.

  • Effective treatment is not possible unless

Introduction and demographics

Approximately 3% to 10% of adults with congenital heart disease (ACHD) will develop pulmonary hypertension (PH).1, 2, 3 The causes of PH in congenital heart disease (CHD) are diverse. For example, patients with septal defects may have pulmonary arterial hypertension (PAH) with vascular remodeling and increased pulmonary vascular resistance (PVR), whereas patients with left-sided lesions may have PH due to pulmonary venous hypertension. In addition to different causes of PH associated with

Physical Examination and Bedside Tests Including 6-minute Walk Testing

The physical examination provides insight regarding the presence, severity, and consequences of PH in patients with CHD. Hepatomegaly, elevated jugular venous pressure, peripheral edema, ascites, and cool extremities are consistent with low-output right heart failure and suggest advanced disease. A pulsatile liver suggests substantial tricuspid regurgitation. Elevated jugular venous pressure is not specific for PAH and is present in many patients with PH due to left heart disease.

The right

Pulmonary Vascular Disease in Patients with Fontan Circulation

More than other forms of CHD, the Fontan circulation demands a low PVR. Because there is no subpulmonary ventricle in the Fontan circulation, chronically elevated central venous pressures drive blood through the lungs. If the transpulmonary gradient increases in the Fontan circulation, then central venous pressure must increase as well. Once the limited capacity to elevate central venous pressure is surpassed, cardiac output and ventricular preload will decrease. The inability to augment

Summary

ACHD-PH is a complex and evolving field. There are an increasing number of medical options, but optimal medication selection and overall therapeutic approach depend on understanding the underlying phenotype of the patient. Compared with non-ACHD patients with PH, the ACHD patient is more likely to have physiology explained by multiple causes/WHO groups, and therefore, requires an even more holistic approach to evaluation and management.

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    Disclosures: Dr E.V. Krieger has received research grant funding from Actelion Pharmaceuticals US Inc. Dr P.J. Leary has nothing to disclose. Dr A.R. Opotowsky has received research grant funding from Actelion Pharmaceuticals US Inc and Merck & Co., Inc. Dr A.R. Opotowsky is supported by the Dunlevie Family Fund.

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