1/58. Congenital hypertrichosis, osteochondrodysplasia, and cardiomegaly: Cantu syndrome.Cantu syndrome (hypertrichosis, osteochondrodysplasia, cardiomegaly) is a rare condition, previously reported in 13 patients. We report on two additional patients with this disorder. One of the patients had pulmonary hypertension of unknown cause which was responsive to steroid therapy. She also had unusual, deep plantar creases, not reported previously in Cantu syndrome. Autosomal recessive inheritance has been suggested previously on the basis of sib recurrence in one family and consanguinity in another. We have performed a segregation analysis based on all reported families to date; the data indicate autosomal recessive inheritance is unlikely. A new dominant mutation or microdeletion syndrome are more likely possibilities, sib recurrence possibly representing gonadal mosaicism.- - - - - - - - - - ranking = 1keywords = hypertension (Clic here for more details about this article) |
2/58. Cardiac involvement in total generalized lipodystrophy (Berardinelli- Seip syndrome).Total generalized lipodystrophy (Berardinelli - Seip syndrome) is a rare hereditary disease characterized by insulin-resistant diabetes mellitus and a small quantity of adipose tissue and is of unknown origin. Common cardiovascular alterations related to this syndrome are cardiac hypertrophy and arterial hypertension. This article reports a case of Berardinelli - Seip syndrome and reviews the literature with special emphasis on the cardiovascular manifestations of this syndrome.- - - - - - - - - - ranking = 1keywords = hypertension (Clic here for more details about this article) |
3/58. Bilateral cystic adenomatoid lung malformation type III--a rare differential diagnosis of pulmonary hypertension in neonates.Congenital cystic adenomatoid lung malformation (CCAM) is a rare disorder characterized by replacement of normal pulmonary tissue with cysts of variable size and distribution. Only sporadic cases of CCAM type III have been described that were stillborn or died in the early neonatal period. To our best knowledge we report the first infant with bilateral CCAM type III that was not ventilator-dependent for the first 10 weeks of life and survived until the age of 5 months. Postnatally the newborn presented with signs of only mild respiratory distress and chest radiograph did not show evidence of cystic lesions. In the following weeks the major clinical symptom was persistent pulmonary hypertension with severe tachypnea, O2 requirement and CO2 retention. Pulmonary hypertension did not respond to HFOV ventilation, NO ventilation or prostacyclin inhalations. CONCLUSIONS: (1) Persistent pulmonary hypertension can be a major clinical feature in neonates with bilateral CCAM type III. (2) A normal chest X-ray does not exclude the possibility of bilateral type III CCAM. (3) Computerized tomography appears to be superior to conventional chest X-ray imaging in detection of small cystic lesions in infants with CCAM type III. (4) In cases of prenatally suspected CCAM type III, parents should be informed that short-term survival is possible.- - - - - - - - - - ranking = 7keywords = hypertension (Clic here for more details about this article) |
4/58. A coconut left atrium 23 years after mitral valve replacement for chronic mitral stenosis.We present the case of a 65 year-old female who was admitted to the hospital because of deterioration of chronic dyspnea. Twenty-three years prior to this admission, mitral valve replacement for chronic mitral stenosis was performed using a Starr-Edwards caged-ball prosthesis. There was severe pulmonary hypertension. On transthoracic echocardiography and on cineradiography, the function of the mitral valve prosthesis was unimpaired. However, cineradiography showed extensive mural calcification of the left atrium; we report this remarkable finding.- - - - - - - - - - ranking = 1keywords = hypertension (Clic here for more details about this article) |
5/58. Regression of primary pulmonary hypertension.A girl who presented in childhood with advanced primary pulmonary hypertension and whose condition later improved is described. This is the first time to our knowledge that documented regression of the disease has been reported.- - - - - - - - - - ranking = 5keywords = hypertension (Clic here for more details about this article) |
6/58. cor triatriatum: echocardiograhic findings.The echocardiographic findings in a patient with cor triatriatum are presented. The preoperative echocardiogram demonstrated an unusual structure behind the mitral valve that moved anteriorly during atrial systole. There was also echocardiographic evidence of abnormalities of mitral valve motion, right ventricular and left atrial enlargement, and pulmonary hypertension. Postoperatively, the unusual echo behind the mitral valve had disappeared and the mitral valve appeared more normal. Left atrial and right ventricular dimension had decreased and the pulmonary valve appeared more normal. Fine diastolic mitral valve fluttering, an abnormal echo behind the anterior mitral valve leaflet moving anteriorly with atrial systole, an abnormal pulmonary valve echogram suggesting pulmonary hypertension, and marked right ventricular and left atrial enlargement were the main echocardiographic findings in our patient.- - - - - - - - - - ranking = 2keywords = hypertension (Clic here for more details about this article) |
7/58. Malignant hypertension and microangiopathic hemolytic anemia.The fourth case found in japan manifesting the features of malignant hypertension and microangiopathic hemolytic anemia (MHA) was reported. In this case, bilateral nephrectomy brought down the blood pressure and relieved MHA. The patient has since been maintained alive and well on hemodialysis up to the time of this report.- - - - - - - - - - ranking = 5keywords = hypertension (Clic here for more details about this article) |
8/58. mitral valve replacement in infancy: a case report.A 1.7 cm. Bjork-Shiley prosthetic valve was successfully inserted into the mitral annulus in a 7-month-old infant weighing 4.5 kilograms. Rapidly progressive pulmonary arteriolar hypertension and heart failure required surgical intervention. Postoperative problems are discussed. This patient's rapid clinical improvement after the operation further indicates that valve replacement is an important therapeutic alternative in carefully selected infants.- - - - - - - - - - ranking = 1keywords = hypertension (Clic here for more details about this article) |
9/58. Pulmonary arterial pulsus alternans secondary to primary pulmonary hypertension.Pulsus alternans of the pulmonary artery without systemic pulsus alternans is uncommon and is associated with multiple diseases. Two cases of pulmonary hypertension with pulmonary arterial pulsus alternans and right-sided heart failure are described. Primary pulmonary hypertension was demonstrated at autopsy in both cases. These two case reports constitute another previously unreported cause for pulsus alternans in the pulmonary circuit.- - - - - - - - - - ranking = 6keywords = hypertension (Clic here for more details about this article) |
10/58. situs inversus, subaortic and subpulmonic stenosis, ventricular septal defect, and single coronary artery.The unusual occurrence of situs inversus totalis, ventricular septal defect, hypertrophic subaortic and subpulmonic stenosis, and single coronary artery in a 38-year-old man is presented. The clinical course was remarkably mild, as documented by data from 23 years of study including four cardiac catheterizations. At age 35 years, however, syncope, chest pain, and marked elevation of right ventricular pressure prompted complete surgical repair of the left and right ventricular outflow tract obstructions and closure of the septal defect. Three years after surgery the patient continues to lead an active life without symptoms. The unusually mild course can be attributed to the natural banding effects of the subpulmonic stenosis which prevented irreversible pulmonary hypertension.- - - - - - - - - - ranking = 1keywords = hypertension (Clic here for more details about this article) |
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