Abstract
A 15-year-old boy with a ventricular septal defect, pulmonary hypertension, Down's syndrome, and extremely thickened media (ETM) of the small pulmonary arteries died of heart failure and pulmonary hypertension 13 years after intracardiac repair. Microscopic examination of lung specimens collected proir to the intracardiac repair and at the time of autopsy revealed that the ETM had remained unchanged and that the arteries connected to the vessels with ETM had become severely thickened. The present case shows that even a small percentage of arteries with ETM can cause pulmonary hypertension, and illustrates one of the mechanisms of how pulmonary hypertension can fail to be resolved after intracardiac repair.
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Moller JH, Patton C, Varco RL, Lillehei CW. Late results after operative closure of isolated ventricular septal defect from 1954 to 1960. Am J Cardiol 1991; 68: 1491–7.
Momma K, Takao A, Ando M, Nakazawa M, Takamizawa K. Natural and postoperative history of pulmonary vascular obstruction associated with ventricular septal defect. Jpn Circ J 1981; 45: 230–6.
Yamaki S, Abe A, Endo M, Tanaka T, Tabayashi K, takahashi T. Surgical indication for congenital heart disease with extremely thickened media of small pulmonary arteries. Ann Thorac Surg 1998; 66: 1560–4.
Yamaki S, Haneda K, Yaginuma G, Akimoto H, Akino Y, Ito T, et al. Lung biopsy diagnosis of operative indication in ventricular septal defect and/or patent ductus arteriosus with severe pulmonary hypertension (Eng abstr). Kyobu Geka 1987; 35: 2143–51.
Suwa N, Takahashi T. Morphological and morphometrical analysis of circulation in hypertension and ischemic kidney. Munchen: Urban & Schwarzenberg 1971: 40–60.
Riemenschneider TA, Emmanouilides MD. Persistent pulmonary hypertension in the new born. In: Adams FH, Emmanouilides GC, Riemenschneider TA, eds. Moss' heart disease in infants, children and adolescents. Fourth Edition. Baltimore: Williams & Wilkins, 1989: 837–41.
Wagenvoort CA, Wagenvoort N, Draulans-Noe Y. Reversibility of plexogenic arteriopathy following banding of the pulmonary artery. J Thorac Cardiovasc Surg 1984; 87: 876–86.
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Maeda, K., Yamaki, S., Nishiyama, M. et al. Pathological lesions causing pulmonary hypertension after closure of a ventricular septal defect. Jpn J Thorac Caridovasc Surg 51, 430–433 (2003). https://doi.org/10.1007/BF02719596
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DOI: https://doi.org/10.1007/BF02719596