A Rare Case of Severe Nontropical Isolated Right Ventricular Endomyocardial Fibrosis.
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SUBMITTER: Laher S
PROVIDER: S-EPMC8299774 | biostudies-literature |
REPOSITORIES: biostudies-literature
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Project description:IntroductionEndomyocardial fibrosis is a neglected tropical disease of unknown etiology and poor prognosis. It is endemic of tropical climates where it is the most common cause of restrictive cardiomyopathy in the second and fourth decades of life. A forme fruste of the disease is thought to be present in temperate climates where the diagnosis remains exceedingly rare.Case presentationWe describe a case of isolated right ventricular endomyocardial fibrosis in a 27-year-old Caucasian man from a temperate climate who presented initially with frank hemoptysis and pulmonary thromboembolic disease. We further describe the approach utilized in the diagnosis, the surgical treatment and the outcome of the disease.ConclusionsWe conclude that endomyocardial fibrosis should be included in the differential diagnosis of apical cardiomyopathies in patients from temperate climates.
| S-EPMC4008263 | biostudies-literature
Project description:A 48-year-old man presented with chronic right sided heart failure. 2D echocardiography revealed the classical features of left ventricular endomyocardial fibrosis with a prominent right ventricular apical aneurysm. Right ventriculography further defined the aneurysm. Cardiac magnetic resonance images revealed a thin-walled, apical aneurysm of right ventricle with multiple septations and marked obliteration of left ventricular apex. A delayed-enhancement sequence after the infusion of contrast, demonstrated a hypersignal in the subendocardium, consistent with the right ventricular involvement of endomyocardial fibrosis. This patient had classical features of left ventricular endomyocardial fibrosis, while on the right side the typical features were missing. This aneurysm may be a passing phase of the natural history of endomyocardial fibrosis before the development of burned out stage. This aneurysm may later develop thrombus, and which may progress to fibrosis and apical obliteration. Endomyocardial fibrosis with right ventricular aneurysm has not heretofore been reported in the medical literature.
| S-EPMC5067796 | biostudies-literature
Project description:BackgroundIsolated right ventricular myocardial infarction (RVMI) due to a recessive right coronary artery (RCA) occlusion is a rare presentation. It is typically caused by right ventricle (RV) branch occlusion complicating percutaneous coronary intervention. We report a case of an isolated RVMI due to flush RCA occlusion presenting via our primary percutaneous coronary intervention ST-elevation myocardial infarction pathway.Case summaryA 61-year-old female smoker with a history of hypercholesterolaemia presented via the primary percutaneous coronary intervention pathway with sudden onset of shortness of breath, dizziness, and chest pain while walking. Transradial coronary angiography revealed a normal left main coronary artery, large left anterior descending artery that wrapped around the apex and dominant left circumflex artery with the non-obstructive disease. The RCA was not selectively entered despite multiple attempts. The left ventriculogram showed normal left ventricle (LV) systolic function. She was in cardiogenic shock with a persistent ectopic atrial rhythm with retrograde p-waves and stabilized with intravenous dobutamine thus avoiding the need for a transcutaneous venous pacing system. A computed tomography pulmonary angiogram demonstrated no evidence of pulmonary embolism while an urgent cardiac gated computed tomography revealed a recessive RCA with ostial occlusive lesion. A cardiac magnetic resonance imaging confirmed RV free wall infarction. She was managed conservatively and discharged to her local district general hospital after 5th day of hospitalization at the tertiary centre.DiscussionThis case describes a relatively rare myocardial infarction presentation that can present with many disease mimics which can require as in this case, a multi-modality imaging approach to establish the diagnosis.
| S-EPMC7850631 | biostudies-literature
Project description:BackgroundPulmonary arterial hypertension is usually fatal due to right ventricular failure and is frequently associated with co-existing left ventricular dysfunction. Endothelin-1 is a powerful pro-fibrotic mediator and vasoconstrictor that is elevated in pulmonary arterial hypertension. Endothelin receptor blockers are commonly used as pulmonary vasodilators, however their effect on biventricular injury, remodeling and function, despite elevated isolated right ventricular afterload is unknown.MethodsElevated right ventricular afterload was induced by progressive pulmonary artery banding. Seven rabbits underwent pulmonary artery banding without macitentan; 13 received pulmonary artery banding + macitentan; and 5 did not undergo inflation of the pulmonary artery band (sham-operated controls).ResultsRight and left ventricular collagen content was increased with pulmonary artery banding compared to sham-operated controls and ameliorated by macitentan. Right ventricular fibrosis signaling (connective tissue growth factor and endothelin-1 protein levels); extra-cellular matrix remodeling (matrix-metalloproteinases 2 and 9), apoptosis and apoptosis-related peptides (caspases 3 and 8) were increased with pulmonary artery banding compared with sham-operated controls and decreased with macitentan.ConclusionIsolated right ventricular afterload causes biventricular fibrosis, right ventricular apoptosis and extra cellular matrix remodeling, mediated by up-regulation of endothelin-1 and connective tissue growth factor signaling. These pathological changes are ameliorated by dual endothelin receptor blockade despite persistent elevated right ventricular afterload.
| S-EPMC4713098 | biostudies-literature
Project description:BackgroundLymphocytic myocarditis is an uncommon condition with a variety of clinical presentations. Isolated involvement of the right ventricle (RV) is very rare. We present a case of a young woman who developed right ventricular dysfunction and arrhythmias as a consequence of this condition, which appeared to be chronic at diagnosis.Case summaryA 26-year-old lady was admitted to hospital following routine echocardiography, requested for screening of pulmonary hypertension in the context of known hypersensitivity pneumonitis. This echocardiogram demonstrated severe right ventricular dilatation and impairment. She was also experiencing atrial fibrillation and non-sustained, symptomatic episodes of ventricular tachycardia. Endomyocardial biopsy revealed lymphocytic myocarditis. She was managed with azathioprine and prednisone, as well as sotalol and apixaban for her atrial fibrillation, and has had no complications in the 12 months since discharge.DiscussionLymphocytic myocarditis isolated to the RV has only been reported in two previous cases, both of which were acute, dramatic presentations. This is the first report of a chronic example of this disease process. Due to her intercurrent immunosuppression, this patient may have been pre-disposed to the condition either by re-activation of a latent viral infection or partial treatment of a true autoimmune lymphocytic myocarditis.
| S-EPMC7501889 | biostudies-literature
Project description:With the present study, we sought to determine the safety of three different endomyocardial biopsy (EMB) access routes in 514 patients admitted for diagnostic workup of heart failure of unknown aetiology. In this retrospective monocentric cohort study, we analysed 514 consecutive patients with heart failure without evidence of significant coronary artery disease or valvular disease undergoing EMB between November 2013 and December 2018, stratified in three access route groups: transradial arterial left ventricular (LV-)EMB (323 patients), transfemoral LV-EMB (138 patients), and transfemoral right ventricular (RV-)EMB (53 patients). Patients undergoing selective transradial LV-EMB were older compared with patients undergoing selective transfemoral LV-EMB or RV-EMB [transradial LV-EMB: 56.0 (45.0/64.0) vs. transfemoral LV-EMB: 53 (42.5/64.5), P = 0.455; transradial LV-EMB: 56 (45.0/64.0) vs. RV-EMB: 53 (42.5/64), P = 0.695] and presented more often in New York Heart Association-functional class III and IV. A total of eight major complications including permanent atrioventricular block requiring pacemaker implantation, pericardial tamponade necessitating pericardiocentesis, stroke and transient cerebral ischaemic attack as well as severe valvular damage, vascular access site complications, and ventricular fibrillation were documented with no significant differences between the groups (8/514, 1.5%). Minor complications such as transient chest pain, non-sustained electrocardiogram abnormalities, and transient atrioventricular block were rare and equally distributed between groups. Transradial LV-EMB is a safe procedure for experienced radial operators and non-inferior compared with transfemoral LV-EMB and RV-EMB. An accurate peri-procedural and post-procedural monitoring and follow-up care should be recommended for all patients undergoing this procedure in order to identify potential complications.
| S-EPMC7754772 | biostudies-literature
Project description:Endomyocardial biopsy (EMB) continues to remain the gold standard for surveillance of rejection post orthotropic heart transplantation (OHT). It can be performed under fluoroscopic or echocardiographic guidance. In the hands of an experienced operator, the complications of EMB are uncommon with <1% chance of any serious acute complications. Most common complications of EMB include access site-related complications, namely, venous thrombosis, carotid cannulation, hematoma, air embolism, and pneumothorax. We present a case of a rare complication of EMB in a patient with OHT causing a coronary sinus to right coronary artery (RCA) fistula.
| S-EPMC7079234 | biostudies-literature
Project description:BackgroundEndomyocardial biopsy (EMB) remains the gold standard for cellular rejection surveillance in heart transplant recipients. Coronary artery fistula formation is a rare late and potentially catastrophic complication of repeated endomyocardial biopsies, without contemporary evidence on incidence or management.Case summaryA 47-year-old male was found to have a fistula between his right ventricle and his left anterior descending artery on an angiogram that was performed as a part of regular screening of coronary allograft vasculopathy. Given the low shunt fraction, asymptomatic nature, and lack of guidelines on definitive management, the patient is undergoing conservative management with regular surveillance.DiscussionCoronary artery fistulas were once thought to be rare complications of repeated EMB, but the true prevalence is likely to be higher than previously believed. Ideal treatment and monitoring is unknown given the relative rarity of the condition.
| S-EPMC8233482 | biostudies-literature
Project description:BackgroundMyxoma is an uncommon disease and its symptoms vary greatly depending on size, location and mobility. Right-sided myxoma, especially right ventricular myxoma, is much rarer, and the symptoms are alien and uncharacteristic. The lack of understandings poses challenges to prompt diagnosis and timely treatment.Case presentationA 44-year-old female patient was diagnosed with giant right ventricular tumor. Right heart failure and systemic congestion caused by right ventricular outflow tract obstruction were observed on this case. Surgery was performed to excise the mass which was measured at 9.5 * 5.0 cm and confirmed as myxoma pathologically.ConclusionsRight-side myxoma is easy to be unnoticed due to its low incident rate and atypical symptoms. Delay in surgical intervention might cause unrecoverable complications. More comprehensive understanding of the symptoms is expected to help improving the diagnose and treat of right-sided myxoma.
| S-EPMC7845124 | biostudies-literature
Project description:BackgroundCardiac haemangioma is a rare primary cardiac tumour. Most patients with cardiac haemangioma have no typical symptoms, and some may present with non-specific manifestations, such as shortness of breath, heart palpitations, or cardiac insufficiency, making it difficult to distinguish cardiac haemangioma from other diseases. We report a case of cardiac haemangioma that present with chest pain. This haemangioma was finally completely excised to relieve the patient's symptoms and a avoid poor prognosis.Case summaryA 14-year-old boy presented with an intermittent and progressive non-exertional chest pain for 2 weeks. Echocardiography showed a space-occupying mass at the right ventricular apex, which was later confirmed by computed tomography angiography and magnetic resonance imaging (MRI). The mass was successfully resected, and postoperative pathology confirmed a cardiac cavernous haemangioma. The patient had an uneventful postoperative recovery at the 8-month follow-up.DiscussionCardiac haemangioma is a benign tumour with no typical clinical manifestations, and very few patients may present with chest pain. Preoperative echocardiography, computed tomography, and MRI are helpful for diagnosis, and surgery can relieve symptoms and may improve the prognosis of patients with cardiac haemangioma.
| S-EPMC8759472 | biostudies-literature