Regression of pituitary tumors, a possible effect of bromergocryptine.

With the advent of the prolactin radioimmunoassay and more sensitive methods of roentgenologic examination, prolactin-secreting pituitary tumors are now being diagnosed with much greater frequency. Definitive treatment has been considered to involve transphenoidal hypophysectomy. The symptoms of hyperprolactinemia including amenorrhea, galactorrhea and infertility can usually be controlled without difficulty by bromergocryptine therapy, but little is known regarding continued tumor growth. Bromergocryptine and other ergot alkaloids have been shown to decrease the production of prolactin and to inhibit the rate of pituitary tumor growth in animal studies. In man, evidence for a similar effect is not as clear. The present study demonstrates tumor regression associated with bromergocryptine therapy in two patients.     

Rest and exercise ventricular function in adults with congenital ventricular septal defects

Rest and exercise right and left ventricular function were compared using equilibrium gated radionuclide angiography in 19 normal sedentary control subjects (mean age 28 years, range 22 to 34) and 34 patients with hemodynamically documented congenital ventricular septal defect (VSD) (mean age 27 years, range 20 to 40). The 34 patients with VSD were divided into 3 groups: those in Group 1 (17 patients) had pulmonary to systemic blood flow ratios of less than 2 to 1; those in Group 2 (12 patients) had prior surgical closure of VSD (mean interval from surgery 17 years, range 9 to 22), and those in Group 3 (5 patients) had Eisenmenger's complex. Gated radionuclide angiography was performed at rest and during each level of graded supine bicycle exercise to fatigue. Heart rate, blood pressure, maximal work load achieved, and right and left ventricular ejection fractions were assessed. The control subjects demonstrated an increase in both the left and right ventricular ejection fractions with exercise (0.70 +/- 0.07 to 0.79 +/- 0.05 and 0.46 +/- 0.06 to 0.57 +/- 0.04; p less than 0.001 for left and right ventricles, respectively). All study groups failed to demonstrate an increase in ejection fraction in either ventricle with exercise. Furthermore, resting left ventricular ejection fraction in Groups 2 and 3 was lower than that in the control subjects (0.59 +/- 0.09 and 0.54 +/- 0.06 versus 0.70 +/- 0.07; p less than 0.001) and resting right ventricular ejection fraction was lower in Group 3 versus control subjects (0.30 +/- 0.07 versus 0.46 +/- 0.06; p less than 0.001). Thus (1) left and right ventricular function on exercise were abnormal in patients with residual VSD as compared with control subjects; (2) rest and exercise left ventricular ejection fractions remained abnormal despite surgical closure of VSD in the remote past; (3) resting left and right ventricular function was abnormal in patients with Eisenmenger's complex; (4) lifelong volume overload may be detrimental to myocardial function.     

Intravenous leiomyomatosis of the uterus with extension into the heart

A 42 year old woman presented with impaired filling of the right atrium and right ventricle two years after removal of uterine leiomyomas. At the time of hysterectomy, intravenous leiomyomatosis was noted, with extension of the tumor into the inferior vena cava. The tumor subsequently extended into the right atrium and coronary sinus, and protruded through the tricuspid orifice. It was successfully removed from the heart, and proved to be histologically benign.     

Echocardiographic evaluation of right ventricular function in complete transposition of the great arteries: angiographic correlates.

Right ventricular function of patients with complete transposition of the great arteries may be impaired. To assess whether the M mode echocardiogram could predict right ventricular function 64 echocardiograms and ventricular angiograms of 62 patients were studied, 33 obtained before repair of the defect (group I) and 31 (including 2 from patients in group I) obtained after the Mustard procedure (group II). Each group was subdivided according to whether a ventricular septal defect or patent ductus arterposus was or had been present preoperatively.The right ventricular preejection period was expressed as the ratio of the observed to the predicted value for a systemic left ventricle. This value was above normal In all groups and was not significantly different in the 13 patients with right bundle branch block. The observed ejection time expressed as a percent of that predicted for a systemic left ventricle correlated with right ventricular ejection fraction (r = 0.47). The ratio of the observed preejection period/ejection time had a higher correlation with right ventricular ejection fraction (r = ? 0.70). Nine patients had a low right ventricular ejection fraction (an ejection fraction of 0.55 or less was considered indicative of poor right ventricular function). These nine patients were all in group II and all had right bundle branch block.Most patients with poor right ventricular function had had a ventricular septal defect or patent ductus arteriosus before repair of the transposition, and postoperatively had angiographic tricuspld regurgitation, right bundle branch block, a preejection period/ejection time ratio greater than 0.55 and a percent predicted ejection time of 96 percent or less. Most patients who had good right ventricular function (ejection fraction greater than 0.55) had none of these findings. Systolic time intervals obtained with M mode echocardiography can be used for the assessment and longitudinal follow-up of right ventricular function in patients with transposition.     

Differentiation of functional and structural pulmonary atresia: Role of aortography

Among certain critically ill neonates with severe tricuspid regurgitation, including those with Ebstein's anomaly of the tricuspid valve, tricuspid valve dysplasia, transient tricuspid regurgitation of the newborn, Uhl's anomaly and hypoplasia of the right ventricle, selective right ventriculography may not distinguish between patients with associated functional and those with structural pulmonary atresia. In patients with a normal pulmonary valve and infundibulum, functional obstruction (nonvisualization of the pulmonary artery on right ventricular angiography when the pulmonary valve is anatomically patent and nonobstructive) results from a combination of massive tricuspid insufficiency and increased perinatal pulmonary vascular resistance. This differentiation between functional and structural pulmonary atresia is essential because of the therapeutic implications.In 33 neonates studied at cardiac catheterization from January 1974 through October 1977 standard hemodynamic measurements and right ventriculograms could not distinguish between functional and structural pulmonary atresia. Retrograde aortography, in the presence of a patent ductus arteriosus, provided excellent visualization of the pulmonary root and valve in these cases and, without exception, pulmonary regurgitation across the valve was observed in the patients with functional pulmonary atresia. Usually, the amount of regurgitated contrast material was dense and both the right ventricle and the right atrium were opacified. In addition, among the patients with functional pulmonary atresia, the pulmonary root and valve appeared wider and more patulous than in the patients with structural pulmonary atresia and normal or mildly increased right ventricular pressure was appreciated.     

右心室肿瘤手术治疗经验

目的：总结右心室肿瘤的手术治疗经验。 方法：回顾性分析我院2009年7月至2016年12月共20例进行右心室肿瘤切除术患者的临床资料。 结果：男性13例,女性7例,平均年龄35.9±22.5(岁),良性肿瘤19例,恶性肿瘤1例,心内膜肿瘤14例,心肌间肿瘤3例,心外膜肿瘤3例。20例患者均采取正中开胸,在体外循环下完成手术,根治性切除15例,姑息性切除5例,同期行三尖瓣系统修复6例,平均转机时间85.6±38.2分钟,平均主动脉阻断时间63.3±32.5分钟,手术相关并发症1例,平均术后住院时间8.3±2.0天。平均随访时间38.9±31.9月,随访过程中恶性肿瘤患者死亡,根治性切除组有1例复发,复发率6.7%(1/15)。右心室心肌间肿瘤与心外膜肿瘤相较于心内膜肿瘤更不易于进行根治性切除 (p=0.026)。姑息性切除术会延长术后住院时间(p=0.001)。 结论：手术是治疗右心室肿瘤的主要方法,肿瘤根治性切除预后较好,姑息性切除和恶性肿瘤预后较差,且姑息性切除术会延长患者术后住院时间。右心室肿瘤切除的同时需要注意保护三尖瓣功能,必要时应积极进行三尖瓣的修复。 关键词：心脏肿瘤;右心室;手术     

The spectrum of right ventricular involvement in inferior wall myocardial infarction: a clinical, hemodynamic and noninvasive study

The clinical experience with 37 patients with acute transmural inferior wall myocardial infarction who were assessed for evidence of right ventricular involvement is reported. On the basis of currently accepted hemodynamic criteria, 29 patients (78%) had evidence suggestive of right ventricular infarction. However, only 5 (20%) of 25 patients demonstrated right ventricular uptake of technetium pyrophosphate on scintigraphy. Two-dimensional echocardiography or isotope nuclear angiography, or both, were performed in 32 patients; 20 studies (62%) showed evidence of right ventricular wall motion disturbance or dilation, or both. Twenty-one patients demonstrated a late inspiratory increase in the jugular venous pressure (Kussmaul's sign). The presence of this sign in the clinical setting of inferior wall myocardial infarction was predictive for right ventricular involvement in 81% of the patients in this study. It is suggested that right ventricular involvement in this clinical setting is common and includes not only infarction but also dysfunction without detectable infarction, which is likely on an ischemic basis.     

Right ventricular myxoma

We recently treated a patient with right ventricular myxoma and a clinical picture of pulmonary embolism and tricuspid valve obstruction who had successful removal of the tumor through the tricuspid valve via a right atriotomy.     

Skeletal response in rats following the implantation of hypercalcemia-producing Leydig cell tumors

The effect of Rice H-500 Leydig cell tumor tissue on bone in rats was assessed by morphometric analysis and tetracycline labeling of the lower femoral metaphyses. The rats in which tumor was implanted showed hypercalcemia, increased osteoclastic bone resorption, inhibition of bone formation with reduction in the bone apposition rate, and a loss in trabecular volume compared with the control rats. There was no evidence of tumor metastasis to bone. The results are consistent with the hypothesis that the Leydig cell tumor secretes a humoral factor capable of causing systemic bone resorption.     

Blood pressure,renin-angiotensin system and urinary kallikrein in a case of juxtaglomerular cell tumor

A right renin-secreting renal juxtaglomerular-cell tumor was found in a 26 year old woman with severe hypertension associated with hypokatemia, proteinuria, normal renal function and raised peripheral venous levels of angiotensin II, plasma renin activity (PRA) and aldosterone. Hypertension had been discovered 55 months earlier, during the third trimester of pregnancy. The infusion of saralasin, a specific angiotensin II-antagonist, produced a marked decrease in arterial pressure from 220/162 to 162/106 mm Hg and a decrease in plasma aldosterone from 48 to 35.4 ng/100 ml, however, PRA did not increase as expected during saralasin administration. Arterial pressure was unchanged during beta-blockade (acebutolol). The tumor was visualized on renal arteriography (radiotranslucent zone 2 cm in diameter) and lateralized by renal vein catheterization (ratio of renin in right renal vein:lower inferior vena cava of 1.41). Peroperative clamping of the right renal pedicle was associated with rapid and parallel decreases of mean arterial pressure from 146 to 82 mm Hg (30 minutes after clamping) and peripheral PRA (half-life of renin 39 minutes).Arterial pressure was 134/90 mm Hg after local excision of the tumor which contained very large amounts of renin (200 times normal), and the presence of an acid-activable form of renin was identified (57 per cent of “total” renin). Numerous mast cells were found within the tumor tissue, and tumor cells contained rhomboidal and spherical secretory granules, thus confirming the existence of the two forms of renin found on biochemical studies. Severe nephroangiosclerosis was found in the extratumoral renal tissue.Urinary kallikrein excretion which was very high before surgery decreased to normal after removal of the tumor.Local excision of tumor is recommended in this type of curable hypertension. A lack of increase of PRA during angiotensin II-blockade may be a useful diagnostic test in the absence of roentgenologic evidence of renin-secreting tumor.     

Intrapericardial pheochromocytoma

A patient with intrapericardial pheochromocytoma is described, and the literature on this subject is reviewed. Iodine 131-labeled metaiodobenzylguanidine scintigraphy was superior to computed tomography in localizing the tumor. This newly introduced method is safe, specific, noninvasive, and may prove to be the method of choice for localizing pheochromocytomas. The patient was prepared for surgery with alpha- and beta-adrenoceptor blocking agents. The tumor was markedly adherent to the posterior wall of the left and right atria. Cardiopulmonary bypass was used to allow excision of the tumor. Hemostasis was attained with difficulty due to marked vascularity of the tumor.     

96例右中叶综合征病因分析

目的探讨右中叶综合征的病因分布规律。方法分析96例临床诊断右中叶综合征患者的病因，并按年龄分组进行比较。结果病因组成炎症占66．7％，肿瘤占17．7％，结核占9．4％。炎症在青年组和中年组间有显著差异（P均〈0．05）；肿瘤及结核在青年组和中年组间、青年组和老年组间均有显著差异（P均〈0．05）。结论右中叶综合征最常见的病因依次为炎症、肿瘤和结核，埘于年龄〉40岁的患者应高度警惕罹患癌症的可能。     

Mechanism of acquired right ventricular outflow tract obstruction in patients with ventricular septal defect: an angiocardiographic study

Right ventricular outflow tract obstruction can complicate the natural history of patients with isolated ventricular septal defect. Although numerous pathologic mechanisms may be responsible for this phenomenon, it is less clear what the frequency is of each of these mechanisms in any cohort of patients studied in a single institution. We identified 20 patients with isolated ventricular septal defect from 1970 to 1981 who acquired a right ventricular outflow tract gradient of 25 mm Hg or more. They ranged in age at the initial catheterization investigation from 4 days to 23 months and at the subsequent study from 13 months to 8 years. Angiographically, 2 mechanisms were implicated in the 20 patients. Hypertrophy of right ventricular anomalous muscle bundles was identified in 19 of the 20 while hypertrophy of a malaligned infundibular septum (that is, classic tetralogy type) was identified in only 1 patient. These data indicate that acquired right ventricular outflow tract obstruction is rarely of the classic tetralogy type.     

Carcinoid heart disease: Clinicopathologic findings and follow-up in 11 cases

Eleven cases of carcinoid heart disease are reported, including two in which a primary, ovarian carcinoid tumor without metastases was extirpated but tricuspid incompetence progressed. Findings on routine physical examinations, right heart catheterization, phonocardiography, electrocardiography, chest roentgenograms, right ventriculography and patho-anatomic investigation are briefly described. The natural history of carcinoid heart disease is commented upon. It is concluded that carcinoid heart disease may be a lethal manifestation of carcinoidosis even in cases in which disseminated tumor growth persists, and that it may have a progressive further course of its own even in cases in which the carcinoid tumor has been successfully surgically removed.     

Detection of radiation cardiomyopathy by gated radionuclide angiography

Twenty-one asymptomatic adults underwent rest and exercise gated radionuclide angiography seven to 20 years after having received mediastinal radiation (2,000 to 7,600 rads) for Hodgkin's disease. None of these patients received cytotoxic chemotherapy. Twelve patients (57 percent) had abnormal left (less than 53 percent at rest and/or greater than 5 percent decrease at peak exercise) and/or right (less than 27 percent at rest and/or greater than 5 percent decrease at peak exercise) ventricular ejection fractions. Previous reports have described myocardial fibrosis occurring late after therapeutic mediastinal radiation; however, the incidence of this occurrence based on clinical follow-up has been low. Rest and exercise radionuclide angiography is a sensitive method for assessing systolic ventricular function and reveals a high prevalence of cardiomyopathy that can be linked to previous radiotherapy.     

Unusual echographic manifestations of right and left heart myxomas.

Although echocardiography has provided a useful noninvasive means for detecting cardiac myxomas, the ultrasound manifestations of these tumors may be variable. We describe our experiences with unusual echographic features encountered in left and right heart myxomas. Thus the left atrial tumor may be manifested predominantly by multiple, discrete, linear echoes behind the mitral valve, the anterior leaflet of which may exhibit an abrupt mid-systolic posterior movement. In right heat myxomatous tumor arising from the septal tricupsid leaflet and adjacent interventricular septum, the echographic characteristics include a cloud of echoes throughout the cardiac cycle in the right ventricular outflow tract which are present in the right ventricle body only during relaxation and are anterior to the tricuspid valve in early diastole. Therefore, discrete linear echoes may be the principal echographic presentation of left atrial myxoma, and special attention should be focused on all areas of the tricuspid valve and right ventricle by ultrasound in patients in whom diagnosis of myxoma is suspected.     

Aneurysm of the atrial septum in tricuspid atresia: Diagnosis during life and therapy

Massive aneurysmal dilatation of the foramen ovale was diagnosed angiocardiographically in a patient with tricuspid atresia. The angiographic findings are distinct for this condition, and the pathogenesis appears to be related to a restrictive atrial communication in the patient with obligatory right to left shunting at atrial level. The anatomic potential for atrial restriction in the patient with tricuspid or pulmonary atresia, intact ventricular septum and diminutive right ventricle necessitates balloon atrial septostomy at the initial diagnostic cardiac catheterization. In the patient with aneurysmal dilatation of the foramen ovale, satisfactory decompression may be achieved by Blalock-Hanlon atrial septectomy, open atrial septectomy or, possibly, balloon septostomy.     

Massive pulmonary embolism associated with a right ventricular myxoma

A 12 year old boy had a massive pulmonary embolism associated with a right ventricular myxoma. This caused complete occlusion of the main trunk of the left pulmonary artery and of a branch of the right pulmonary artery supplying the basal area of the lower lobe of the right lung. The patient died despite two surgical attempts to remove the tumor clots. To our knowledge this constitutes the first report of a massive pulmonary artery embolism associated with a right ventricular myxoma.     

Complete transposition of the great arteries and coarctation of the aorta

Thirty-two patients with complete transposition of the great arteries (TGA) and coarctation of the aorta (C of A) were seen at The Hospital for Sick Children, Toronto, Canada, between 1963 and 1983. Three patients had only mild C of A and have not required coarctectomy (Group I); 29 had a severe form of C of A (Group II). Two patients in Group I and 21 in Group II had a ventricular septal defect. Subaortic obstruction was present in 5 patients in Group II. The mechanisms included anterior deviation of the infundibular ventricular septum, anomalous right ventricular muscle bundles, and abnormal ventriculoinfundibular fold. Five patients in Group II had a hypoplastic right ventricle. Coarctectomy was performed in 25 patients, and 5 died (20% mortality rate). Sixteen patients had repair for TGA (13 Mustard, 2 Jatene, 1 Rastelli), and 2 died (12% mortality rate). Life-table analysis shows that only 68% of the patients with TGA and C of A survived the first month of life. The 5-year survival in this group was 57%. In the same period, 94% of patients with uncomplicated TGA survived the first month of life and the 5-year survival rate was 89%.     

Two-dimensional echocardiography in the detection of noneffusive cardiac involvement by intrathoracic neoplasms

Two-dimensional echocardiographic (2DE) findings in four patients with predominantly noneffusive manifestations of intrathoracic neoplasms are presented. In cases Nos. 1 and 2 tumor masses were identified in the left atrioventricular groove area at a time when the left-heart border was obscured by a large pleural effusion on chest x-ray examination. Case No. 3 demonstrated distortion of right ventricular anatomy by a compressing extrinsic mass lesion. In these three cases the neoplasm itself or the complicating pleural effusion provided additional echocardiographic windows for visualizing the heart. Case No. 4 demonstrated dilation of the main pulmonary artery secondary to tumor compression of the left pulmonary artery with regression of the dilation following resection. The relative applicability of 2DE and M-mode techniques and their clinical relevance in tumor patients are delineated.