Pulmonary hypertension, congenital heart

FIGURE 8.1 Comparisons of survival in primary pulmonary hypertension, congenital heart defects, and collagen vascular disease (unpublished data). (CHD, congenital heart disease CTD, connective tissue disease.) (From Ref. 4.)... 

By 1993 we had explored the clinical applications of inhaled nitric oxide in over 200 children with pulmonary hypertension or respiratory failure (Fig. 1). The majority of these patients suffered from pulmonary hypertensive congenital heart disease and were under 1 year of age, reflecting the bias toward early surgical repair of congenital heart defects at Children s Hospi-... 

Other reported side effects include vomiting, salivation, lacrimation, shivering, skin rash, and an interaction with thyroid preparations that may lead to hypertension and tachycardia. Ketamine also may raise intracranial pressure and elevate pulmonary vascular resistance, especially in children with trauma or congenital heart disease. Increases in intraocular pressure also may occur, and vigilance is required if ketamine is used in ocular surgery. 

Unlabeled Uses Cardiopulmonary bypass surgery hemodialysis pulmonary hypertension associated with acute respiratory distress syndrome, systemic lupus erythematosus, or congenital heart disease refractory CHF severe community-acquired pneumonia... 

The first combined heart-lung transplant was performed in 1981 at Stanford University. Combined heart-lung donors need to satisfy both the requirements already described separately. Combined heart-lung transplant is recommended in patients with congenital problems affecting these organs, pulmonary hypertension and/or cystic fibrosis. The recipients for the combined transplant are recommended to be less than 55 years old. Survival rates are 79, 66, and 54% at 1 month, 1 year and 3 years, respectively, after transplantation. 

Bizzarre M, Gross I, Bizzarre M. Inhaled nitric oxide for the postoperative management of pulmonary hypertension in infants and children with congenital heart disease. Cochrane Database Syst Rev. 2005 CD005055. 

Fuse S, Kamiya T. Plasma thromboxane Bj concoitration in pulmonary hypertension associated with congenital heart disease. Circulation 1994 90 2952-5... 

Despite the high success rates, 20% of neonates fail to respond to treatment. Factors associated with poor response inclnde sepsis, pnenmonia, PDA, congenital heart disease, pulmonary hypertension, meconium aspiration, and pulmonary hypoplasia. - Whether retreatment of RDS with surfactant after resolution of these factors is nsefnl is currently unknown. 

Varan B, Tokel K, Yilmaz G. Malnutrition and growth failure in cyanotic and acyanotic congenital heart disease with and without pulmonary hypertension. Arch Dis Child 1999 81 49-52. 

Rosenzweig EB, Kerstein D, Barst RJ, et al. Long-term prostacyclin for pulmonary hypertension with associated congenital heart defects. Circulation 1999 99(14) 1858—65. 

FIGURE I Age analysis of 200 consecutive patients who received inhaled nitric oxide. The majority of the patients were less than 1 year old and had congenital heart disease (CHD) or persistent pulmonary hypertension of the newborn (PPHN). 

We challenged 12 patients with congenital heart disease and pulmonary hypertension preoperatively in the cardiac catheterization laboratory and nine postoperative patients (after cardiopulmonary bypass) with a 2-min infusion of 10 M acetylcholine into the pulmonary artery. The nine postoperative patients received a 15-min trial of inhaled nitric oxide following the infusion of acetylcholine. The results are shown in Figs. 4 and 5. The decrease in pulmonary artery pressure and resistance in the preoperative patients in response to acetylcholine was markedly attenuated postoperatively. The percentage reductions in pulmonary artery pressure preoperatively were 27% 4% and 9% 2% P < 0.003) postoperatively. Similarly, pulmonary vascular resistance decreased by 46% 5% compared to 11% 4% P < 0.002). However, the response to inhaled nitric oxide in the postoperative patients was marked vasodilation contrasted with the blunted effect of acetylcholine. Mean pulmonary artery pressure (34.4 2.6 versus... 

Alternatively, the increased pulmonary vascular resistance may be due to an increase in a circulating vasoconstrictor such as endothelin or thromboxane, both of which have been reported to be elevated in children with congenital heart disease following cardiopulmonary bypass. It is noteworthy that the excretion of thromboxane has been found to be elevated during a pulmonary hypertensive crisis ° and that in animal studies inhaled nitric oxide is effective in reversing the pulmonary vasoconstriction induced by thromboxane mimetics. ... 

Journois, D., Pouard, P., Mauriat, P., Malhere, T., Vouhe, P., and Safran, D. (1994). Inhaled nitric oxide as a therapy for pulmonary hypertension after operations for congenital heart defects. J. Tborac. Cardiovasc. Surg. 107, 1129-1135. 

Yoshibayashi, M., Nishioka, K., Nakao, K., et al. (1991). Plasma endothelin concentrations in patients with pulmonary hypertension associated with congenital heart defects. Evidence for increased production of endothelin in pulmonary circulation. Circulation 84, 2280-2285. 

Komai, H., Adatia, I., Elliott, M. J. De Laval, M. R., and Haworth, S. G. (1993). Increased plasma levels of endothelin-1 after cardiopulmonary bypass in patients with pulmonary hypertension and congenital heart disease. ]. Thorac. Cardiovasc. Surg. 106, 473-478. 

Hopkins, R. A., Bull, C.,De Leval, M. R., Haworth, S. G.,and Stark, J. (1991). Pulmonary hypertensive crisis following surgery for congenital heart defects in young children. Eur. J. Cardiovasc. Thorac. Surg. 5, 628-634. 

Complicated congenital heart disease (including pulmonary hypertension)... 

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