A case of lipomatous hypertrophy of right ventricle

Lipomatous hypertrophy is a condition that is being increasingly diagnosed with the advent of newer modalities. A middle aged lady was referred to us with recent onset dyspnea and palpitations. Echocardiography revealed diffuse thickening of the right ventricular free wall and outflow tract. Endomyocardial biopsy revealed this as a lipomatous hypertrophy involving the right ventricle. This is the first reported case of lipomatous hypertrophy involving the right ventricle.     

Hypertrophie lipomateuse du septum interatrial

Lipomatous hypertrophy of the heart is a benign rare tumor characterized by fatty tissue deposits in the interatrial septum. Its diagnosis during life is mostly seen incidental. We report the case of a 74-year-old female patient, in which a cardiac mass protruding in the right atrium was diagnosed by transthoracic echocardiography. Transoesophageal echocardiography examination detected an echogenic infiltration and hypertrophy of the interatrial septum, sparing the foramen ovale. The diagnosis of lipomatous hypertrophy of the interatrial septum is confirmed by MRI, which shows a well limited and not encapsulated tumor, appearing in increased signal density on T1-weighted images, and that decreases with fat-saturated sequences. This tumor did not enhance with the administration of contrast material. The benign and lipomatous nature of the lesion is confirmed, thus making the use of histology unnecessary.     

Early Detection of a Cavopulmonary Tumor Embolus with the Use of Transesophageal Echocardiography

Pulmonary tumor embolization from renal cell carcinoma is associated with severe cardiopulmonary morbidity and high perioperative mortality rates. We report the case of a 71-year-old woman who presented with right-sided abdominal pain. Magnetic resonance images revealed a mass originating from the upper pole of the right kidney and extending into the infrahepatic portion of the inferior vena cava. Transesophageal echocardiography was continuously used to monitor the mass during intended radical nephrectomy and tumor resection. When the right kidney was mobilized, intracaval thrombus detached and migrated to the patient''s right atrium, causing severe hemodynamic instability. After emergent sternotomy and during the initiation of cardiopulmonary bypass, the mass was no longer echocardiographically detectable in the heart; it was soon removed completely from the left pulmonary artery. The mass was a renal cell carcinoma.We recommend the use of transesophageal echocardiography as an efficient diagnostic tool in the early detection of pulmonary tumor embolization during the resection of renal cell carcinoma that involves the inferior vena cava.     

Cardiac Sarcoidosis Presenting as Constrictive Pericarditis

In patients with cardiac sarcoidosis, the sarcoid granulomas usually involve the myocardium or endocardium. The disease typically presents as heart failure with ventricular arrhythmias, conduction disturbances, or both. Constrictive pericarditis has rarely been described in patients with sarcoidosis: we found only 2 reports of this association.We report the case of a 57-year-old man who presented with clinical and hemodynamic features of constrictive pericarditis, of unclear cause. He was admitted for treatment of recurrent pleural effusion. After a complicated hospital course, he underwent pericardiectomy. His clinical and hemodynamic conditions improved substantially, and he was discharged from the hospital in good condition. The pathologic findings, the patient''s clinical course, and his response to pericardiectomy led to our diagnosis of cardiac sarcoidosis presenting as constrictive pericarditis. In addition to the patient''s case, we discuss the nature and diagnostic challenges of cardiac sarcoidosis. Increased awareness of this disease is necessary for its early detection, appropriate management, and potential cure.     

Orthodeoxia platypnea syndrome in a patient with lipomatous hypertrophy of the interatrial septum due to long-term steroid use

We describe an unusual case of orthodeoxia platypnea syndrome exacerbated by right ventricular inflow obstruction due to iatrogenic steroid-induced adipose deposition in cardiac tissues. A 68-year-old man on long-term prednisone therapy for eosinophilic pneumonia presented with progressive dyspnea worsened by bending forward. By using pulse oximetry, he was noted to have positional hypoxemia. Transthoracic echocardiogram demonstrated normal right-sided pressures but severe right to left shunting through a patent foramen ovale. Transesophageal echocardiogram showed a large patent foramen ovale, severe lipomatous hypertrophy of the interatrial septum, and massive adipose deposition in the pericardium causing compression of the right ventricular inflow tract. The patient underwent percutaneous closure of the patent foramen ovale, which resulted in the resolution of symptoms and hypoxemia. This case is unique because long-term steroid use resulted in reverse Lutembacher physiology and clinical orthodeoxia platypnea syndrome by inducing lipomatous hypertrophy of the interatrial septum and compression of the right atrium.     

Unusual Presentation of Listerial Myocarditis and the Diagnostic Value of Cardiac Magnetic Resonance

Listeria monocytogenes is an infrequent cause of bacterial myocarditis. Myocarditis without evidence of endocarditis is even rarer. Management in such cases involves early diagnosis, antibiotic therapy, and emergency treatment of arrhythmias.We report the case of a 47-year-old man who presented with features of acute ST-segment-elevation myocardial infarction complicated by ventricular tachycardia that necessitated urgent electrical cardioversion. Contrast-enhanced cardiac magnetic resonance images revealed hypertrophy, necrosis, and a mass that was determined to be an abscess caused by L. monocytogenes. Antibiotic treatment led to resolution of the listerial myocarditis.In addition to reporting our patient''s case, we discuss the comparative advantages of cardiac magnetic resonance versus transthoracic echocardiography in characterizing myocarditis, upon presentation and in follow-up evaluation.     

Right Ventricular Sarcoidosis: Is It Time for Updated Diagnostic Criteria?

A 55-year-old woman with a history of complete heart block, atrial flutter, and progressive right ventricular failure was referred to our tertiary care center to be evaluated for cardiac transplantation. The patient''s clinical course included worsening right ventricular dysfunction for 3 years before the current evaluation. Our clinical findings raised concerns about arrhythmogenic right ventricular cardiomyopathy. Noninvasive imaging, including a positron emission tomographic scan, did not reveal obvious myocardial pathologic conditions. Given the end-stage nature of the patient''s right ventricular failure and her dependence on inotropic agents, she underwent urgent listing and subsequent heart transplantation. Pathologic examination of the explanted heart revealed isolated right ventricular sarcoidosis with replacement fibrosis. Biopsy samples of the cardiac allograft 6 months after transplantation showed no recurrence of sarcoidosis. This atypical presentation of isolated cardiac sarcoidosis posed a considerable diagnostic challenge. In addition to discussing the patient''s case, we review the relevant medical literature and discuss the need for updated differential diagnostic criteria for end-stage right ventricular failure that mimics arrhythmogenic right ventricular cardiomyopathy.     

Regression of Left Ventricular Mass after Implantation of the Sutureless 3f Enable Aortic Bioprosthesis

Left ventricular hypertrophy in aortic stenosis is considered a compensatory response for the maintenance of systolic function but a risk factor for cardiac morbidity and death. We investigated the degree of left ventricular mass regression after implantation of the sutureless Medtronic 3f Enable^® Aortic Bioprosthesis.We studied 19 patients who, from May 2010 through July 2011, underwent isolated aortic valve replacement with the 3f Enable bioprosthetic valve, with clinical and echocardiographic follow-up at 6 months. The mean age was 77.1 ± 5.1 years (range, 68–86 yr); 14 patients were women (73.7%); and the mean logistic EuroSCORE was 15.4% ± 11.8%. Echocardiography was performed preoperatively, at discharge, and at 6 months'' follow-up. The left ventricular mass was calculated by means of the Devereux formula and indexed to body surface area.The left ventricular mass index decreased from 146.1 ± 47.6 g/m² at baseline to 118.1 ± 39.8 g/m² at follow-up (P=0.003). The left ventricular ejection fraction did not change significantly. The mean transaortic gradient decreased from 57.3 ± 14.2 mmHg at baseline to 12.3 ± 4.6 mmHg at discharge and 12.2 ± 5.3 mmHg at follow-up (P <0.001), and these decreases were accompanied by substantial clinical improvement. No moderate or severe paravalvular leakage was present at discharge or at follow-up.In isolated aortic stenosis, aortic valve replacement with the 3f Enable bioprosthesis results in significant regression of left ventricular mass at 6 months'' follow-up. However, this regression needs to be verified by long-term echocardiographic follow-up.     

Defect closure in the lipomatous hypertrophied atrial septum with the amplatzer muscular ventricular septal defect closure device: A case series

Variations in the anatomy of the atrial septum can confound the transcatheter closure of atrial septal defects and patent foramen ovale. While lipomatous hypertrophy of the atrial septum is not frequently encountered, attempts to use standard atrial septal occlusion devices in such patient can lead to malapposition and continued shunt. Some operators have suggested that atrial septal hypertrophy is a relative contraindication to transcatheter‐based closure techniques. We present a novel technique exploiting the features of the Amplatzer muscular ventricular septal defect occluder to treat atrial septal defects in the setting of lipomatous hypertrophy of the atrial septum in two patients. © 2011 Wiley‐Liss, Inc.     

Left Ventricular Aneurysm Repair with Use of a Bovine Pericardial Patch

Left ventricular aneurysm, which can impair systolic function, has a reported incidence of 10% to 35% in patients after myocardial infarction. In a 58-year-old woman who had a history of myocardial infarction, we excised a large left ventricular aneurysm and restored left ventricular geometry with use of a bovine pericardial patch. The aneurysm''s characteristics and the patient''s preoperative left ventricular ejection fraction of 0.25 had indicated surgical intervention. The patient had an uneventful postoperative course, and her left ventricular ejection fraction was 0.50 to 0.55 on the 4th postoperative day. This case illustrates the value of surgical treatment for patients who have a debilitating left ventricular aneurysm.     

Lipomatous Hypertrophy of the Interatrial Septum

Lipomatous hypertrophy of the interatrial septum is a clinicopathological entity defined as an accumulation of fatty tissue exceeding 15 mm in thickness in the interatrial septum above the fossa ovalis. It is clinically associated with atrial electric abnormalities, interference with venous return, and sudden death. Transesophageal echocardiography is superior to transthoracic echocardiography for the study of the interatrial septum. We describe one case of lipomatous hypertrophy of the interatrial septum in which transesophageal echocardiography was used, we believe for the first time, to study the interatrial septal anatomy.     

Cone Reconstruction of Atypical Ebstein Anomaly Associated with Right Ventricular Apical Hypoplasia

Cone reconstruction for tricuspid valve repair has revolutionized the surgical treatment of Ebstein anomaly. We present the case of a 58-year-old woman with atypical Ebstein anomaly and right ventricular apical hypoplasia who was spared from palliative shunt physiology by our use of cone reconstruction.Compared with other techniques, cone reconstruction of the tricuspid valve more closely replicates normal valvular anatomy and function. This surgical procedure can be applied to many anatomic variations of Ebstein anomaly, as in our patient''s apparently unique instance of atypical Ebstein anomaly with right ventricular apical hypoplasia.     

Getting to a Man's Heart through His Colon

A 69-year-old man presented with a progressively enlarging pulsatile mass in the left side of his chest. Because of a history of an ischemic cardiomyopathy, he had been randomized in 2003 to undergo coronary artery bypass grafting with a Dor procedure, as part of the Surgical Treatment for Ischemic Heart Failure (STICH) trial.Our patient''s imaging studies, including a thoracic computed tomogram and transthoracic echocardiogram, were now of concern for left ventricular pseudoaneurysm. He was taken immediately for surgical exploration. Purulent material, with empyema, extended from the anterior chest wall through the chest cavity into the mediastinum, with communication into the pericardial space. Notably, there was no compromise of the left ventricular cavity, and there was no pseudoaneurysm. The chest was copiously irrigated before closure. The epicardial patch placed 10 years earlier in the STICH trial was not thought to be the nidus of the abscess and was therefore not removed.Three months later, the patient presented again, this time with hemorrhagic shock and bleeding from his left anterior thoracotomy site, which we then re-entered. He was found to have a left ventricular pseudoaneurysm with disruption of the ventricular apex. The epicardial felt-and-Dacron patch, placed 10 years previously during his Dor procedure, was found to be infected with Clostridium difficile and was removed. The left ventricular apex was repaired.Whereas C. difficile bacteremia is rare, the seeding of prosthetic cardiac material with delayed presentation, as in this case, is extraordinarily uncommon.     

Right Ventricular Outflow Tract Obstruction Caused by Double-Chambered Right Ventricle Presenting in Adulthood

Congenital heart diseases that cause obstruction of the right ventricular outflow tract are often difficult to diagnose. We report the case of a 49-year-old man who presented with long-standing shortness of breath on exertion. Imaging revealed right ventricular outflow tract obstruction caused by a double-chambered right ventricle, and he was referred for surgical correction. This case emphasizes both the detailed perioperative evaluation that is needed when diagnosing adults who present with manifestations of congenital heart disease and a method of successful surgical correction that resulted in symptom resolution.     

An unusual case of cyanotic heart disease in a patient with patent foramen ovale and right ventricular hypertrophy

An unusual case of cyanosis and patent foramen ovale/atrial septal defect with right-sided septal hypertrophy is presented. Mild right ventricular dysfunction led to predominant right-to-left shunting. The atrial defect was surgically closed. The patient is from a family with hypertrophic cardio-myopathy;she is negative for the gene abnormality.     

Screening for Fabry Disease in Left Ventricular Hypertrophy: Documentation of a Novel Mutation

Background

Fabry disease is a lysosomal storage disease caused by enzyme α-galactosidase A deficiency as a result of mutations in the GLA gene. Cardiac involvement is characterized by progressive left ventricular hypertrophy.

Objective

To estimate the prevalence of Fabry disease in a population with left ventricular hypertrophy.

Methods

The patients were assessed for the presence of left ventricular hypertrophy defined as a left ventricular mass index ≥ 96 g/m² for women or ≥ 116 g/m² for men. Severe aortic stenosis and arterial hypertension with mild left ventricular hypertrophy were exclusion criteria. All patients included were assessed for enzyme α-galactosidase A activity using dry spot testing. Genetic study was performed whenever the enzyme activity was decreased.

Results

A total of 47 patients with a mean left ventricular mass index of 141.1 g/m² (± 28.5; 99.2 to 228.5 g/m²] were included. Most of the patients were females (51.1%). Nine (19.1%) showed decreased α-galactosidase A activity, but only one positive genetic test − [GLA] c.785G>T; p.W262L (exon 5), a mutation not previously described in the literature. This clinical investigation was able to establish the association between the mutation and the clinical presentation.

Conclusion

In a population of patients with left ventricular hypertrophy, we documented a Fabry disease prevalence of 2.1%. This novel case was defined in the sequence of a mutation of unknown meaning in the GLA gene with further pathogenicity study. Thus, this study permitted the definition of a novel causal mutation for Fabry disease - [GLA] c.785G>T; p.W262L (exon 5).     

Normal Pregnancy and Birth Despite Ruptured Sinus of Valsalva Aneurysm: Another Case for the Record

Sinus of Valsalva aneurysm is a rare cardiac abnormality. Ruptured sinus of Valsalva aneurysms in pregnancy are of course rarer still. We present a case in which an aneurysm ruptured into the right ventricular outflow tract during pregnancy.In 2012, a 26-year-old Chinese woman, in the 18th week of pregnancy and with no apparent evidence of cardiac problems, was diagnosed with atrioventricular septal defects and a sinus of Valsalva aneurysm that had ruptured into her right ventricular outflow tract. After an uncomplicated full-term pregnancy, she gave birth to a healthy baby boy by cesarean section. Fifty days postpartum, the patient underwent surgical repair of the ruptured aneurysm and other cardiac defects. Her surgical outcome was good. As of May 2013, the patient and her baby were healthy.Ruptured sinus of Valsalva aneurysm in pregnancy can be asymptomatic, and women with such a rupture can have a normal full-term pregnancy and give birth to healthy babies. Cesarean section is preferable for pregnant women with ruptured sinus of Valsalva aneurysm because the hemodynamic changes associated with labor can aggravate the aneurysm. Surgical repair should be performed as soon as the patient''s condition allows.     

Left Atrial Myxoma Causing Coronary Steal: An Atypical Cause of Angina

Cardiac myxomas are rare primary cardiac tumors that usually present with dyspnea or manifestations of systemic embolization. Coronary steal is a rare phenomenon of unbalanced blood flow that is seen primarily in patients who have undergone coronary artery bypass grafting and have subclavian artery stenosis.We report the case of a 72-year-old woman who presented with fatigue, weakness, and exertional chest heaviness and had abnormal results on a cardiac stress test. The results of coronary angiography showed no obstructive coronary artery disease but revealed a large intracardiac left atrial mass that was supplied by 2 anomalous coronary arteries. The patient underwent successful ligation of the anomalous coronary arteries and resection of the mass, which was histologically an atrial myxoma. The patient''s symptoms resolved, and results of a repeat cardiac stress test were normal.To our knowledge, this is the first report of a highly vascularized atrial myxoma that caused coronary steal with objective evidence of ischemia, and with subsequent resolution after resection of the mass and ligation of the anomalous coronary arteries.     

Intravenous Leiomyomatosis with Intracardiac Extension: Echocardiographic Study and Literature Review

Uterine leiomyomatosis is a common disease in women; however, intravenous leiomyomatosis with intracaval and intracardiac tumor extension is rare. We sought to analyze the clinical and echocardiographic features of intracardiac leiomyomatosis.From January 2003 through July 2012, 7 women (age range, 24–59 yr) underwent surgical resection of histopathologically diagnosed intracardiac leiomyomas at our hospital. Most of the patients had histories of hysterectomy or uterine leiomyoma. We retrospectively analyzed their preoperative echocardiograms. We found that the tumors had no stalks, did not adhere to the wall of the right side of the heart, were highly mobile, and moved back and forth in the right atrium near the tricuspid orifice. All tumors originated from the inferior vena cava and had borders well demarcated from that structure''s wall. Most of the masses extended into the inferior vena cava and right atrium through the right internal and common iliac veins. Computed tomograms revealed pelvic tumors and contiguous filling defects in 6 patients.When echocardiograms reveal a right-sided cardiac mass that originates from the inferior vena cava, particularly in women who have a history of hysterectomy or uterine leiomyoma, intracardiac leiomyomatosis should be suspected. If the mass has no stalk and freely moves within the inferior vena cava and right-sided cardiac chambers without attachment to the endothelial surface or endocardium, intracardiac leiomyomatosis should be diagnosed. We discuss our findings and briefly review the relevant medical literature.     

Right ventricular subendocardial infarction in a patient with pulmonary hypertension,right ventricular hypertrophy,and normal coronary arteries

Right ventricular infarction is usually associated with coronary artery disease and concomitant left ventricular infarction. Isolated right ventricular subendocardial necrosis was discovered at autopsy in a 52-year-old woman with pulmonary hypertension, right ventricular hypertrophy, and normal coronary arteries, who died with septicemia 41 days after mitral valve replacement. This represents the first well-documented report of isolated right ventricular subendocardial infarction associated with normal coronary arteries.