A Long-Term Survivor With Alveolar Capillary Dysplasia

Abstract

A patient with alveolar capillary dysplasia has survived more than 56 months with medical therapy. Intrauterine exposure to metformin potentially modified the severity of disease. In combination with other agents, endothelin receptor antagonists and amlodipine have been key medications in lowering pulmonary arterial pressure and managing right heart failure. (Level of Difficulty: Beginner.).

Keywords: ACD, alveolar capillary dysplasia; PH, pulmonary hypertension; echocardiography; iNO, inhaled nitric oxide; pulmonary hypertension; right-sided heart catheterization.

Graphical abstract

Figure 1

Chronological Summary of Medical Care (A) Key events (upper section); (B) Serial measurements of the end-systolic eccentricity index, an echocardiographic estimate of the ratio of systolic pulmonary to systolic systemic arterial pressure (normal: 1.00). Waveforms of tricuspid valve regurgitation were rarely adequate to estimate the right ventricular systolic pressure by echocardiography. (C) Serial measurements of B-type natriuretic peptide, a gauge of right heart function (normal: <100 pg/ml). (D) Medications. (E) Age.

Figure 2

Histology Samples Demonstrating a Mild Variant of Alveolar Capillary Dysplasia (A) There is medial muscularization of the intralobular arterioles (a) with adjacent dilated venules (b). (B) A thickened pulmonary arteriole (c) is surrounded by an adjacent bronchial venule (d, misaligned pulmonary vein) and bronchiole (e). In both images, the capillary bed is diminished, and there is heterogeneous alveolar septal thickening. The lack of homogenous septal thickening demonstrates a milder pattern in this disease process.