Heart and Vessels

, Volume 34, Issue 3, pp 470–476 | Cite as

Pulmonary arterial compliance is a useful predictor of pulmonary vascular disease in congenital heart disease

  • Jun MuneuchiEmail author
  • Yoshie Ochiai
  • Naoki Masaki
  • Seigo Okada
  • Chiaki Iida
  • Yuichiro Sugitani
  • Yusuke Ando
  • Mamie Watanabe
Original Article


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.


Congenital 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.


  1. 1.
    Wagenvoort CA, Nauta J, van der Schaar PJ, Weeda HW, Wagenvoort N (1968) The pulmonary vasculature in complete transposition of the great vessels, judged from lung biopsies. Circulation 38:746–754CrossRefGoogle Scholar
  2. 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
  3. 3.
    Yamaki S, Ogata H, Haneda K, Mohri H (1990) Indication for open lung biopsy in patients with ventricular septal defect and/or patent ductus arteriosus with pulmonary hypertension. Heart Vessels 5:166–171CrossRefGoogle Scholar
  4. 4.
    Yamaki S, Tezuka F (1976) Quantitative analysis of pulmonary vascular disease in complete transposition of the great arteries. Circulation 54:805–809CrossRefGoogle Scholar
  5. 5.
    Yamaki S, Abe A, Tabayashi K, Endo M, Mohri H, Takahashi T (1998) Inoperable pulmonary vascular disease in infants with congenital heart disease. Ann Thorac Surg 66:1565–1570CrossRefGoogle Scholar
  6. 6.
    Maeda K, Yamaki S, Yokota M, Murakami A, Takamoto S (2004) Hypoplasia of the small pulmonary arteries in total anomalous pulmonary venous connection with obstructed pulmonary venous drainage. J Thorac Cardiovasc Surg 127:448–456CrossRefGoogle Scholar
  7. 7.
    Maeda K, Yamaki S, Kado H, Asou T, Murakami A, Takamoto S (2004) Hypoplasia of the small pulmonary arteries in hypoplastic left heart syndrome with restrictive atrial septal defect. Circulation 110:II139-146CrossRefGoogle Scholar
  8. 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
  9. 9.
    Lankhaar JW, Westerhof N, Faes TJ, Marques KM, Marcus JT, Postmus PE, Vonk-Noordegraaf A (2006) Quantification of right ventricular afterload in patients with and without pulmonary hypertension. Am J Physiol Heart Circ Physiol 291:H1731–1737CrossRefGoogle Scholar
  10. 10.
    Muneuchi J, Nagatomo Y, Watanabe M, Joo K, Onzuka T, Ochiai Y, Joo K (2016) Relationship between pulmonary arterial resistance and compliance among patients with pulmonary arterial hypertension and congenital heart disease. J Thorac Cardiovasc Surg 152:507–513CrossRefGoogle Scholar
  11. 11.
    Saouti N, Westerhof N, Postmus PE, Vonk-Noordegraaf A (2010) The arterial load in pulmonary hypertension. Eur Respir Rev 19:197–203CrossRefGoogle Scholar
  12. 12.
    Lankhaar JW, Westerhof N, Faes TJ, Gan CT, Marques KM, Boonstra A, van den Berg FG, Postmus PE, Vonk-Noordegraaf A (2008) Pulmonary vascular resistance and compliance stay inversely related during treatment of pulmonary hypertension. Eur Heart J 29:1688–1695CrossRefGoogle Scholar
  13. 13.
    Tedford RJ, Hassoun PM, Mathai SC, Girgis RE, Russell SD, Thiemann DR, Cingolani OH, Mudd JO, Borlaug BA, Redfield MM, Lederer DJ, Kass DA (2012) Pulmonary capillary wedge pressure augments right ventricular pulsatile loading. Circulation 125(2):289–297CrossRefGoogle Scholar
  14. 14.
    Goldner J (1938) A modification of the masson trichrome technique for routine laboratory purposes. Am J Pathol 14:237–243Google Scholar
  15. 15.
    Tonelli AR, Alnuaimat H, Mubarak K (2010) Pulmonary vasodilator testing and use of calcium channel blockers in pulmonary arterial hypertension. Respir Med 104:481–496CrossRefGoogle Scholar
  16. 16.
    Mahapatra S, Nishimura RA, Sorajja P, Cha S, McGoon MD (2006) Relationship of pulmonary arterial capacitance and mortality in idiopathic pulmonary arterial hypertension. J Am Coll Cardiol 47:799–803CrossRefGoogle Scholar
  17. 17.
    Douwes JM, Roofthooft MT, Bartelds B, Talsma MD, Hillege HL, Berger RM (2013) Pulsatile haemodynamic parameters are predictors of survival in paediatric pulmonary arterial hypertension. Int J Cardiol 168:1370–1377CrossRefGoogle Scholar
  18. 18.
    Takatsuki S, Nakayama T, Ikehara S, Matsuura H, Ivy DD, Saji T (2017) pulmonary arterial capacitance index is a strong predictor for adverse outcome in children with idiopathic and heritable pulmonary arterial hypertension. J Pediatr 180:75–79.e2CrossRefGoogle Scholar
  19. 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. 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. 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

Copyright information

© Springer Japan KK, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Department of PediatricsJapan Community Healthcare Organization Kyushu HospitalKitakyushuJapan
  2. 2.Department of Cardiovascular SurgeryJapan Community Healthcare Organization Kyushu HospitalKitakyushuJapan
  3. 3.Department of Cardiovascular Surgery, Graduate School of MedicineTohoku UniversitySendaiJapan

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