Alterations in pulmonary blood flow distribution in heart diseases with pulmonary hypertension studies by radioisotope (131-I-MAA) scanning.
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Morphometric analysis of small pulmonary arterial changes was performed in three patients with different congenital heart disease with pulmonary hypertension: congenital mitral stenosis (MS), ventricular septal defect (VSD) and transposition of the great arteries (TGA). The material was biopsy or autopsy lung specimens, all having the same degree of elevated pulmonary arterial pressure. Medial thickness was determined by the method of Suwa and Takahashi, and the degree of intimal changes was quantified using the index of pulmonary vascular disease (IPVD) by Yamaki and Tezuka. It was demonstrated that the medial thickening of the small pulmonary arteries was the strongest in the patient with congenital MS, moderate in VSD, and the weakest in TGA, with statistically significant differences between each pair of these three conditions. Interestingly, the order of severity for intimal changes was reversed: it was the severest in TGA and the mildest in congenital MS. We surmise that in patients with TGA, medial hypertrophy is suppressed by sustained vasodilation resulting from the high oxygen saturation of pulmonary arterial blood, while in congenital MS, the media undergoes the severest hypertrophy because of the low oxygen saturation. We also conclude that in TGA intimal changes readily develop in the presence of attenuated media, while in congenital MS, the thickened media seems to prevent intimal changes. From a clinical viewpoint, these results urge us to recommend early surgical intervention in TGA and VSD where severe intimal changes can develop in the absence of extreme medial thickening. It may not be appropriate to extend operation on patients with congenital MS, since strongly thickened media can trigger vasospasms and medial necrosis.
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Heart disease is the most important nonobstetric cause of maternal death; however, most young women with heart disease do well during pregnancy. If the physician is uncertain of the effects of pregnancy on a particular heart condition, needless restrictions may be imposed. The main hazards are: pulmonary edema when it occurs suddenly in mitral stenosis; pulmonary hypertension (because pulmonary vascular disease tends to be exacerbated by pregnancy); infective endocarditis (this is rare); and fulminating peripartum cardiomyopathy. The practical management of the pregnant patient with various concomitant heart conditions (congenital heart disease, pulmonary hypertension, rheumatic heart disease, anticoagulants and artificial valves, constrictive pericarditis, kyphoscoliosis, Marfan's syndrome, mitral prolapse, hypertrophic cardiomyopathy, dilated cardiomyopathy, infective endocarditis, and arrhythmias) is discussed. An absolute indication for therapeutic abortion is severe pulmonary vascular disease; discretionary indications include 'chronic thromboembolic pulmonary hypertension,' cardiomyopathies (depending on the hemodynamic disturbance), and Marfan's syndrome.
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The relation between pulmonary vascular pathology and hemodynamics in 81 patients with congenital heart disease complicated by pulmonary hypertension was studied. Slight to marked increase of mean pulmonary pressure (MPAP) was seen in 5 of them, but no vascular lesion was found. 59 of the 81 cases had marked increase of MPAP and Grade 1-2 pulmonary vascular lesions, and cyanosis was seen clinically. Postoperative effects were satisfactory, indicating that not all patients with cyanosis contraindicated surgical treatment. The rest (17) of the 81 patients had most marked increase of MPAP and Grade 3-4 pulmonary vascular lesions with good postoperative results, but long-term results await follow-up observations. It is thought that Grade 4 pulmonary vascular lesion should be considered as a contraindication for corrective surgery.
In a prospective symptom-oriented study, patients with (n = 81) or without (n = 206) digoxin toxicity were not discernible on their serum digoxin concentration (SDC) because of a large overlap between toxic and non-toxic groups. There was, however, a significant difference between the mean values of the groups (p < 0,01). Serum creatinine, the presence or absence of ischemic heart disease, and/or chronic pulmonary heart disease were significantly different (both p < 0,025) between toxic and non-toxic groups. It is concluded that a decision on digitalis toxicity should be made by a synopsis of the influencing factors in the individual case. Dosage, serum creatinine and cardiac status seem to be the most important factors to be taken into account. Lack of agreement between SDC and digoxin effects could be demonstrated in differences in "T 1/2" between SDC and the normalisation of prolonged PQ-time after digoxin withdrawal. The inability of the SDC to describe all alterations important for digitalis effects and side effects seems to be that it does neither mirror the glycoside concentration at the receptor site, nor the changes of receptor affinity, nor the changes of (Na+ + K+)-ATPase activity.
In congenital heart disease (CHD) with pulmonary hypertension (PH) resulting from high pulmonary flow, we sometimes experience patients whose PH continues even after the normalization of flow following surgery. Preoperative identification of these patients is difficult even using direct hemodynamic measurements at cardiac catheterization. The purpose of this study is to interpret the state of the pulmonary vascular bed more precisely using a new theoretical approach and to present the criteria available for avoiding residual PH. During preoperative routine catheter examination in CHD patients with PH we applied the Windkessel model to the pulmonary circulation and calculated the diastolic time constant (Td) of the pulmonary circulation. Td of Group 1 (0.50 +/- 0.03 sec), 6 patients who manifested residual PH after surgery, was higher than that of group 2 (0.23 +/- 0.14 sec), 42 patients with normalized pulmonary artery pressure after surgery. Furthermore, the relationships between Td and pulmonary blood flow (Qp) or pulmonary to systemic flow ratio (Qp/Qs) were very sensitive for distinguishing between these two groups. Group 1 was located in the left-upper quadrant of the graph of Td vs Qp or Td vs Qp/Qs relationships, whereas group 2 was located in the right-lower quadrant. The relationships between Td and Qp or Qp/Qs were considered to reflect the pathological change in the pulmonary vasculature more precisely. As a consequence, they would be very useful in understanding the state of the pulmonary vascular bed and avoiding residual PH.
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