1/46. Unusual phenotypic observations associated with a rare HbH disease genotype (- -Med/alphaTSaudialpha): implications for clinical management.A single patient with a rare Haemoglobin H (HbH) disease genotype (- -Med/alphaTSaudialpha) was observed to have exceptionally high levels of HbH (> 60%) and paradoxically high total haemoglobin levels. Studies of haematological parameters, blood biochemistry and oxygen transport properties revealed a severe functional anaemia, associated with marked erythropoietic stimulation and a markedly raised cardiac output. This rare case illustrates the complexity of interactions that may be associated with the clinical course of HbH disease, highlighting that haematological parameters alone may lead to spurious evaluation of clinical status. Issues related to the therapeutic management of unusual cases are raised.- - - - - - - - - - ranking = 1keywords = cardiac (Clic here for more details about this article) |
2/46. Coil embolization therapy in congenital coronary arterial fistulas.Three pediatric patients (8.5 years, 3 years, and 1 month) presented with congenital coronary arterial fistulas. In all cases the fistulas entered into the right side of the heart (main pulmonary artery, n = 1; right ventricle, n = 2). In the first patient, the fistula and an open ductus arteriosus were closed during the same intervention. The second patient presented with a single left coronary ostium and residual shunt from the coronary artery system to the right ventricle after surgery. The third child had pulmonary atresia with intact ventricular septum and a fistula from the left coronary artery to the right ventricle. The fistulas in all patients were managed with coil occlusion. fistula occlusion was documented with angiocardiography.- - - - - - - - - - ranking = 0.27868105119933keywords = heart (Clic here for more details about this article) |
3/46. Extension of adrenocortical carcinoma into the right atrium--echocardiographic diagnosis: a case report.BACKGROUND: adrenocortical carcinoma is a rare, highly malignant tumor. Cardiac involvement of the tumor is very rare. echocardiography facilitates the evaluation of the cardiac involvement of the tumor. CASE PRESENTATION: We describe a patient with an adrenal tumor. Transthoracic echo showed its extension into the right atrium. Accordingly, a combined abdominal and cardiac operation was performed, monitored by transesophageal echocardiography. CONCLUSION: This case highlights the importance of echocardiography in revealing the cardiac involvement by this tumor and in planning the operative procedure.- - - - - - - - - - ranking = 3keywords = cardiac (Clic here for more details about this article) |
4/46. Ruptured aneurysm of the sinus of valsalva into the right atrium. An uncommon congenital heart defect.rupture of an aneurysm of the sinus of valsalva is an uncommon heart defect. A continuous murmur may be the first clinical sign of this rupture. Additional imaging techniques, and in the first place echocardiography, can be used to confirm the diagnosis. We present a case where, at the age 53 years, the diagnosis was made of a ruptured sinus of valsalva into the right atrium, with a typical windsock image on echocardiogram. Although several cases of a ruptured sinus of valsalva are reported in the literature, it is still an underdiagnosed condition because it is frequently missed during physical examination.- - - - - - - - - - ranking = 1.3934052559967keywords = heart (Clic here for more details about this article) |
5/46. Asymptomatic isolated congenital left ventricular muscular diverticulum in an adult: a case report.Congenital ventricular diverticulum is a very rare malformation in adults. We describe a 21-year-old male with a congenital muscular left ventricular diverticulum in the inferior wall. The lesion was suspected on two-dimensional transthoracic echocardiography; transesophageal echocardiography allowed clear detection of the diverticulum as well of mild mitral valve prolapse. The diagnosis was confirmed by cardiac catheterization. There were no other thoracoabdominal or cardiac anomalies, the patient was asymptomatic, and surgery was not deemed necessary.- - - - - - - - - - ranking = 2keywords = cardiac (Clic here for more details about this article) |
6/46. Noncompaction of the ventricular myocardium: report of two cases with bicuspid aortic valve demonstrating poor prognosis and with prominent right ventricular involvement.Noncompaction of the ventricular myocardium is a rare, unclassified cardiomyopathy due to an arrest of myocardial morphogenesis. The characteristic echocardiographic findings consist of multiple, prominent myocardial trabeculations and deep intertrabecular spaces communicating with the left ventricular (LV) cavity. The disease typically involves the LV myocardium, but right ventricular (RV) involvement is not uncommon. The clinical manifestations include heart failure (HF) signs, ventricular arrhythmias and cardioembolic events. Noncompacted myocardium may occur as an isolated cardiac lesion, as well as it can be in association with congenital anomalies. We describe two illustrative cases of noncompaction of the ventricular myocardium, a 19-year-old male with bicuspid aortic valve and progressive worsening of HF, and a 61-year-old male with marked RV involvement in addition to LV apical involvement, both with the typical clinical and echocardiographic features of the disease.- - - - - - - - - - ranking = 1.2786810511993keywords = cardiac, heart (Clic here for more details about this article) |
7/46. Uncommon presentation and surgical correction of unroofed coronary sinus syndrome.A 59-year-old man with signs and symptoms of congestive heart failure, occurring a few months after an infective episode, underwent cardiac investigations revealing severe biventricular dysfunction, persistent left superior vena cava with almost completely unroofed coronary sinus, and critical stenosis of the proximal right coronary artery. Surgical correction of the congenital malformation associated with revascularization of the right coronary allowed a prompt recovery of clinical conditions and ventricular function.- - - - - - - - - - ranking = 1.2786810511993keywords = cardiac, heart (Clic here for more details about this article) |
8/46. A rare localization in right-sided endocarditis diagnosed by echocardiography: a case report.BACKGROUND: Right-sided endocarditis occurs predominantly in intravenous drug users, patients with pacemakers or central venous lines and with congenital heart diseases. The vast majority of cases involve the tricuspid valve. CASE PRESENTATION: A case of a 31-year-old woman with intravenous drug abuse who had a right-sided vegetation attached to the muscular bundle of the right ventricle is presented. Transthoracic echocardiography revealed a vegetation in the right ventricular outflow tract. Transesophageal echocardiography clearly showed that the 1.8 cm vegetation was not adherent to the pulmonary valve but attached to a muscular bundle. CONCLUSIONS: Our case points to an unusual location of right-sided endocarditis in intravenous drug users. It confirms that TTE remains an easy and highly sensitive first-line examination for the diagnosis of right-sided endocarditis.- - - - - - - - - - ranking = 0.27868105119933keywords = heart (Clic here for more details about this article) |
9/46. Cardioverter defibrillator implantation in a child with isolated noncompaction of the ventricular myocardium and ventricular fibrillation.isolated noncompaction of the ventricular myocardium is a rare unclassified cardiomyopathy and is thought to be due to arrest of myocardial morphogenesis. In fetal life, it is characterized by an excessively prominent trabecular meshwork and deep intratrabecular recesses, and occurs in the left ventricle in the absence of structural heart disease. echocardiography provides evidence for the diagnosis. The noncompacted ventricular myocardium may be accompanied by depressed ventricular function, systemic embolism, wolff-parkinson-white syndrome, left bundle branch block, and ventricular arrhythmia. Although onset of symptoms is frequently delayed until adulthood, symptomatic children have a poor prognosis. In this report, we describe a case of 6-year-old girl who had a history of recurrent syncope. Transthoracic echocardiographic examination showed a localized prominent trabeculation and deep intratrabecular recesses at the inferoapical region of the left ventricle. She had several episodes of ventricular fibrillation which was refractory to pharmacological treatment. An implantable cardioverter defibrillator (ICD) was successfully operated three times during follow-up.- - - - - - - - - - ranking = 0.27868105119933keywords = heart (Clic here for more details about this article) |
10/46. A case of congenital pulmonary vein stenosis in an adult.A 35-year-old Japanese woman, complaining of dyspnea after her first delivery, was diagnosed as having primary pulmonary hypertension. Continuous intravenous prostacyclin resulted in an improvement in her cardiac function, 6-min walk and new york Heart association class, before she died of pulmonary hypertension crisis during further evaluation for pulmonary transplantation. Since the autopsy findings revealed that all 4 pulmonary veins were extremely stenotic due to hypoplasia, she was diagnosed as having had congenital pulmonary vein hypoplasia with stenosis.- - - - - - - - - - ranking = 1keywords = cardiac (Clic here for more details about this article) |
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