Pulmonary arterial compliance is a useful predictor of pulmonary vascular disease in congenital heart disease
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Histopathological assessment of the pulmonary arteries is crucial to determine the surgical indications in patients with congenital heart disease (CHD) and intractable pulmonary vascular disease (PVD). We aimed to clarify whether pulmonary hemodynamic parameters can predict PVD in patients with CHD and pulmonary arterial hypertension (PAH) We performed histopathological evaluations of lung specimens and cardiac catheterizations in 27 patients with CHD–PAH. We divided these patients into the patients with and without PVD, and compared pulmonary hemodynamic parameters including pulmonary arterial compliance (Cp) between two groups. Age at lung biopsy was 4 (2–7) months. There were 16 patients with trisomy 21. Cardiac diagnosis included ventricular septal defect in 16, atrial septal defect in 5, atrioventricular septal defect in 4, and others in 2. There were 11 patients with histopathologically proven PVD (Heath–Edwards classification grade ≥ 3 in 5; the index of PVD ≥ 1.1 in 3; extremely thickened media in 6; hypoplasia of the pulmonary arteries in 3). Cp in the patients with PVD was significantly lower than that in patients without PVD (0.99 [0.74–1.42] vs 1.56 [1.45–1.88], p = 0.0047), although there was no significant difference in the ratio of systemic to pulmonary blood flow, pulmonary arterial pressure, and resistance between two groups. A Cp cutoff value of < 1.22 ml/mmHg m2 as a predictor of PVD yielded a sensitivity and a specificity of 93% and 64%, respectively. Pulmonary arterial compliance can be a predictor of PVD among patients with CHD–PAH.
KeywordsCongenital heart disease Pulmonary hypertension Pulmonary vascular disease Compliance Histopathology
Compliance with ethical standards
Conflicts of interest
There is no conflict of interest for all authors. No funding was secured for this study.
- 2.Schuuring MJ, van Riel AC, Vis JC, Duffels MG, van Dijk AP, de Bruin-Bon RH, Zwinderman AH, Mulder BJ, Bouma BJ (2015) New predictors of mortality in adults with congenital heart disease and pulmonary hypertension: Midterm outcome of a prospective study. Int J Cardiol 181:270–276CrossRefGoogle Scholar
- 8.Moller JH, Neal WA (1990) Fetal, neonatal, and, infant cardiac disease. In: Emmanouilides GC (eds) Persistent pulmonary hypertension in the neonate. Appleton & Lange, Norwalk, pp77– 86Google Scholar
- 14.Goldner J (1938) A modification of the masson trichrome technique for routine laboratory purposes. Am J Pathol 14:237–243Google Scholar
- 19.Ghio S, Crimi G, Pica S, Temporelli PL, Boffini M, Rinaldi M, Raineri C, Scelsi L, Pistono M, Totaro R, Guida S, Oltrona Visconti L (2017) Persistent abnormalities in pulmonary arterial compliance after heart transplantation in patients with combined post-capillary and pre-capillary pulmonary hypertension. PLoS One 12(11):e0188383CrossRefGoogle Scholar
- 20.Ghio S, Crimi G, Temporelli PL, Traversi E, La Rovere MT, Cannito A, Vizza D, Scelsi L, Raineri C, Guazzi M, Oltrona Visconti L (2018) Haemodynamic effects of an acute vasodilator challenge in heart failure patients with reduced ejection fraction and different forms of post-capillary pulmonary hypertension. Eur J Heart Fail 20(4):725–734CrossRefGoogle Scholar
- 21.Yamaki S, Yasui H, Kado H, Yonenaga K, Nakamura Y, Kikuchi T, Ajiki H, Tsunemoto M, Mohri H (1993) Pulmonary vascular disease and operative indications in complete atrioventricular canal defect in early infancy. J Thorac Cardiovasc Surg 106:398–405Google Scholar