Pulmonary vascular occlusive disease is a serious complication in a significant number of patients with congenital heart defects.8 Surgical correction of congenital heart defects is now possible during the first two years of life with low mortality and this has been demonstrated to produce regression of the pulmonary vascular disease.8.59Various studies have demonstrated the defects in which pulmonary vascular lesions occur and those lesions in which progression of changes is likely to occur.8-21 Continuing development of diagnostic techniques may soon allow us to detect individuals at particular risk to develop pulmonary vascular disease.
Moore KL. The Developing Human. Toronto: W. B. Saunders Company, 1973, pp. 239–80.
Boyden EA. The developing bronchial arteries in a fetus of the twelfth week. Am J Anat 1970; 129:357–68.
Auer J. The development of the human pulmonary vein and its major variations. Anat Rec 1948; 101:581–94.
Elliot FM, Reid LM. Some new facts about the pulmonary artery and its branching pattern. Clin Radiol 1965; 16:193–8.
Reid LM. The pulmonary circulation: remodelling in growth and disease. Am Rev Respir Dis 1979; 119:531–46.
Hislop A, Reid L. Pulmonary arterial development during childhood: branching pattern and structure. Thorax 1973; 28:129–35.
Nadas AS, Fyler DF. Pediatric Cardiology. Philadelphia: WB Saunders Company, 1972, pp. 684.
Friedli B, Kidd BS, Mustard WT, Keith JD. Ventricular septal defect with increased pulmonary vascular resistance. Am J Cardiol 1974; 33:403–9.
DuShane JW, Krongrad E, Ritter DG, McGoon DC. The fate of raised pulmonary vascular resistance after surgery in ventricular septal defect. In: Rowe RD, Kidd BSL, eds. The Child with Congenital Heart Disease after Surgery. Mount Kisco, New York: Futura, 1976 p. 299–314.
Hallidie-Smith KA, HollmanA, Cleland WP, Bentall HH, Goodwin JF. Effects of surgical closure of ventricular septal defects upon pulmonary vascular disease. Br Heart J 1966; 31:246–60.
Hoffman HE, Rudolph AM. The natural history of ventricular septal defects in infancy. Am J Cardiol 1965; 16:634–53.
Castaneda AR, Zamora R, Nicoloff DM et al. High pressure, high resistance ventricular septal defect: surgical results of closure through right atrium. Ann Thorac Surg 1971; 12:29–38.
Maron BJ, Redwood DR, Hirschfield FW Jr et al. Postoperative assessment of patients with ventricular septal defect and pulmonary hypertension. Response to intense upright exercise. Circulation 1973; 48:864–74.
Reid LM, Stevenson JG, Coleman EN et al. Moderate to severe pulmonary hypertension accompanying patent ductus arteriosus. Br Heart J 1964; 26:60–5.
Newfield EA, Sher M, Paul MH, Higashi N. Pulmonary vascular disease in complete atrio-ventricular canal defect. Am J Cardiol 1977; 39:721–6.
Marcelletti C, McCoon DC, Mair DD. The natural history of truncus arteriosus. Circulation 1976; 54:108–11.
Besterman E. Atrial septal defect with pulmonary hypertension. Br Heart J 1961; 23:587–98.
Viles PM, Ongley PA, Titus JL. The spectrum of pulmonary vascular disease in transposition of the great arteries. Circulation 1969; 40:31–41.
NewfieldEA, Paul MH, Muster AJ, Idriss FS. Pulmonary vascular disease in complete transposition of the great arteries. A study of 200 patients. Am J Cardiol 1974; 34:75–82.
Waldman JD, Paul MH, Newfeld EA, Muster AJ, Idriss FF. Transposition of the great arteries with intact ventricular septum and patent ductus arteriosus. Am J Cardiol 1977; 39:232–8.
Berman W Jr,Whitman V, Pierce WS, Waldhausen JA. The development of pulmonary vascular obstructive disease after successful Mustard operative in early infancy. Circulation 1978; 58:181–5.
Paul MH, Miller RA, Potts WJ. Long-term results of aortic pulmonary anastomosis in tetralogy of Fallot: an analysis of the first 100 cases 11 to 13 years after operation. Circulation 1961; 23:525–33.
Heath D, Edwards JE. The pathology of hypertensive pulmonary vascular disease. Circulation 1958, 18:533–47.
Wagenvoort CA. Lung biopsy specimens in the evaluation of pulmonary vascular disease. Chest 1980;77:614–25.
Short DS. Postmortem pulmonary arteriography with special reference to the study of pulmonary hypertension. J Fac Radiol (Lond) 1956; 8:118–31.
Davies G, Reid L. Growth of the alveoli and pulmonary arteries in children. Thorax 1970; 25:669–811.
Haworth SG, Reid L. A morphometric study of regional variation in infants with pulmonary hypertension and congenital heart defect. A justification of lung biopsy. Br Heart J 1978; 40:825–31.
Rabinovitch M, Keane JF, Fellows KE, Castaneda AR, Reid L. Quantitative analysis of the pulmonary wedge angiogram in congenital heart defects. Correlation with hemodynamic data and morphometric findings in lung biopsy tissue. Circulation 1981; 63:152–64.
Rabinovitch M, Sanders SP, Castaneda AR, Reid L. Morphometric analysis of lung biopsy tissue in candidates for Fontan-type surgical procedures. Am J Cardiol 1981; 47:447 (Abst).
Hislop A, Haworth SG, Shinebourne EA et al. Quantitative structural analysis of pulmonary vessels in isolated ventricular septal defect in infancy. Br Heart J 1975; 37:1014–21.
Haworth SG, Sauer U, Buhlmeyer K, Reid L. Development of the pulmonary circulation in ventricular septal defect: a quantitative structural study. Am J Cardiol 1977; 40:781–8.
Johnson JB, Felter ML, West JR, Cournand A. The relationship between electrocardiographic existence of right ventricular hypertrophy and pulmonary arterial pressure in patients with chronic pulmonary disease. Circulation 1950; 1:536–50.
Chou T, Masangkay MP, Young R, Conway GF, Helm RA. Simple quantitative vector cardiographic criteria for the diagnosis of right ventricular hypertrophy. Circulation 1973; 48:1262–7.
Weidman WH, DuShane JW, Kincaid OW. Observations concerning progressive pulmonary vascular obstruction in children with ventricular septal defects. Am Heart J 1963; 65:148–54.
Iverson RE, Linde IM, Kegel S. The diagnosis of progressive pulmonary vascular disease in children with ventricular septal defects. J Pediatr 1966; 68:594–600.
Silverman NH, Snider AR, Rudolph AM. Evaluation of pulmonary hypertension by M-mode echocardiography in children with ventricular septal defect. Circulation 1980; 61:1125–32.
Rabinovitch M, Fisher KC, Treves S. Quantitative thallium-201 myocardial imaging in assessing right ventricular pressure in patients with congenital heart defects. Br Heart J 1981; 45:198–205.
Stevenson G, Kawabori I, Guntheroth WG. Non-invasive detection of pulmonary hypertension in-patent ductus arteriosus by pulsed Doppler echocar-diography. Circulation 1979; 60:355–9.
Stocker FP, Wilhoss OS, Nadas AS. Oxygen consumption in infants with heart disease. J Pediatr 1972;80:43–51.
Tarnow J, Hess W. Pulmonary hypertension and pulmonary oedema caused by intravenous ketamine. Anesthetist 1978; 27:486–7.
Rosenthal A, Nathan DG, Marty AT et al. Acute hemodynamic effects of red cell volume reduction in polycythemia of cyanotic congenital heart disease. Circulation 1970; 42:297–307.
Vogel JH, McNamara DC, Blount SG Jr. Role of hypoxia in determining pulmonary vascular resistance in infants with ventricular septal defect. Am J Cardiol 1967; 20; 346–9.
Keane JF, Ellison RC,Rudd M, Nadas AS. Pulmonary blood flow and left ventricular volumes with transposition of the great arteries and intact ventricular septum. Br Heart J 1973; 35:521–6.
Jones ODH, Shore DF, Rigby ML et al. The use of tolazoline hydrochloride as a pulmonary vasodilator in potentially fatal episodes of pulmonary vasoconstriction after cardiac surgery in children. Circulation 1981; 64:134–99.
Mair DP, Ritter DG, Ongley PA, Helmholz HF Jr. Hemodynamics and evaluation for surgery of patients with complete transposition of the great arteries and ventricular septal defect. Am J Cardiol 1971; 28:632–40.
Vogel JHK, Grover RF, Jamieson G, Blount SG Jr. Long-term physiological observation in patients with ventricular septal defect and increased pulmonary vascular resistance. Adv Cardiol 1974; 11:108–21.
Nihill MR, McNamara DG. Magnification of pulmonary wedge angiography in the evaluation of children with congenital heart disease and pulmonary hypertension. Circulation 1978; 58:1094–1106.
Rabinovitch M, Castaneda AR, Reid L. Lung biopsy with frozen section as a diagnostic aid in patients with congenital heart defects. Am J Cardiol 1981; 47:77–84.
Castaneda AR, Lamberti J, Sade RM, Williams RG, Nadas AS. Open heart surgery during the first three months of life. J Thorac Cardiovasc Surg 1974; 68:719–31.
Mair DD, McGoon DC. Surgical correction of atrioventricular canal during the first year of life. Am J Cardiol 1977; 40:66–9.
Stark J, DeLeval MR, Waterston DJ, Graham GR, Bonham-Carter RE. Corrective surgery of transposition of the great arteries in the first years of life. Results in 63 infants. J Thorac Cardiovasc Surg 1974; 67:673–81.
Whitman V, Ellis NG. Pulmonary hemodynamics after repair of left-to-right shunts associated with pulmonary artery hypertension. In: Rosenthal A, Sonnenbliek EH, Lesen M, eds. Postoperative Congenital Heart Disease, New York: Grune & Stratton, 1975: 111–8.
Cartmill TB, Dushne JW, McGoon DC et al. Results of repair of ventricular septal defect. J Thorac Cardiovasc Surg 1966; 52:486–99.
Wheeler J, George BL, Mulder DG, Jarmakani JM. Diagnosis and management of postoperative pulmonary hypertensive crisis. Circulation 1979; 60:1640–3.
Rubin LJ, Peter RH. Hemodynamics at rest and during exercise after oral hydralazine in patients with cor pulmonale. Am J Cardiol 1981; 47:116–22.
Fuster, V, Giuliani ER, Brandenburg RO, Weidman WH, Edwards ED. The natural history of idio-pathic pulmonary hypertension. Am J Cardiol 1981; 47:422 (Abst.)
Jones AM, Howitt G. Eisenmenger syndrome in pregnancy. Br Med J 1965; 1:1627–31.
Pitts JA, Crosby WM, Basta LL. Eisenmenger’ s syndrome in pregnancy. Does heparin prophylaxis improve maternal mortality rate? Am Heart J 1977; 93:321–6.
Rabinovitch M, Keane JF, Norwood WI, Castaneda AR, Reid L. Vascular structure in lung tissue obtained at biopsy correlated with pulmonary hemodynamic findings after repair of congenital heart defects. Circulation 1984; 69:655–67.
Wood P. The Eisenmenger syndrome or pulmonary hypertension with reversed central shunt. Br Med J 1958; 2:701–9, 755-62.
Gotsman MS, Beck W, Barnard CN, Schrirer V. Haemodynamic studies after repair of ventricular septal defect. Br Heart J 1969; 31:63–8.
Lillehei CW, Anderson RC, Wang Y. Clinical and hemodynamic changes after closure of ventricular septal defects. JAMA 1968; 205:822–7.
Friedman WF, Heiferman MF. Clinical problems of postoperative pulmonary vascular disease. Am J Cardiol 1982; 50:631–6.
About this article
Cite this article
Burrows, F.A., Rabinovitch, M. The pulmonary circulation in children with congenital heart disease: morphologic and morphometric considerations. Can Anaesth Soc J 32, 364–373 (1985). https://doi.org/10.1007/BF03011341
- hypertension pulmonary: aetiology
- prevention and control