Diagnosis Of Right Ventricular Cardiomyopathy Research Articles

498 A missed and misdiagnosed case of pericardial agenesis

Abstract Introduction Pericardium is a fibroserous sac surrounding the heart and the roots of the great vessels. Pericardial agenesis which is a rare condition can be complete or partial and the most common form is absence of left hemipericardium. Pericardial agenesis is often an incidental finding, as it is usually asymptomatic. Moreover patients presenting with symptoms can be missed diagnosed, because it can be difficult to visualise the pericardium clearly with imaging modalities. Herein we present a misdiagnosed pericardial agenesis case. Case Report A 49 years old male was admit to our cardiology clinic for confirmation, and follow-up of a previous diagnosis of right ventricular cardiomyopathy (CMP). He had been previously diagnosed based on transthoracic and transesophageal echocardiography findings 13 years ago. Since then he had been attending routine follow-ups, he had also underwent several imagining modalities even cardiac magnetic resonance (CMR) to confirm the diagnosis of right ventricular CMP. No arrhythmia had been detected in Holter monitoring. He had no dyspnea, or other specific signs and symptoms. His medical and family histories were otherwise unremarkable. A 12-lead electrocardiogram (ECG) showed normal sinus rhythm without having specific abnormalities. The chest X-ray revealed laevorotation of the heart (Fig A). The transthoracic echocardiography showed a laterally displaced left ventricle (LV) apex, and an enlarged right ventricle (RV) with normal systolic function (Fig B). Coronary computed tomography angiography (CCTA) revealed normal coronary arteries, leftward displacement of the heart with mild RV dilatation. The pericardium was only visible around right atrium (Fig C). CMR demonstrated mild RV dilatation (end-diastolic volume 167ml) with reasonable systolic function (EF 50%), normal LV dimensions with normal systolic function (Fig D). The absence of pericardium at both left and right sides, except around right atrium, was confirmed, whereas there was no additional finding such as wall motion abnormality. Therefore the patient was diagnosed with partial pericardial agenesis and scheduled for follow-ups. Conclusion The pericardial agenesis which is a rare disorder is generally benign. Although MRI is believed to be the gold standard technique, it might not be able to delineate entire pericardium, because of various artefacts or paucity of surrounding fat. Therefore physicians should be aware of the indirect signs on various imaging modalities like excessive laevorotation of the heart, as direct recognition can be tricky, and the patient can be easily missed and/or misdiagnosed. Our patient was asymptomatic. His pericardial defect was almost total and this abnormality hadn’t been recognized in his previous CMR or echocardiograpy, leading to a misdiagnosis of CMP. Complete or unilateral absence of the pericardium is considered to be benign, surgical pericardioplasty may only be considered for highly symptomatic patients. Abstract 498 Figure.

108 ACUTE RIGHT VENTRICULAR CARDIOMYOPATHY IN PATIENTS WITH SEVERE SEPSIS

Introduction There is an increasing awareness that the cardiovascular pathophysiology of sepsis extends beyond vasodilatation and distributive shock. Acute left ventricular systolic dysfunction is seen in 40–60% of patients admitted to critical care who require organ support. The exact distribution, timing and aetiology of this phenomenon remain unclear as does the therapeutic implications. Although often affecting the left ventricle, cases describing synchronous biventricular or isolated right ventricular dysfunction have been described. This septic cardiomyopathy appears to be fully reversible in survivors. We describe three cases admitted to our intensive care unit who were demonstrated to have an acute isolated dilated right ventricular cardiomyopathy using trans thoracic echocardiography. Methods and results 3 patients admitted to the Adult ICU were echoed on admission to the ICU, all with severe sepsis and multi organ failure. Two required invasive ventilation, all three required inotrope and vasopressor support, with two requiring haemofiltration. All patients had CT contrast pulmonary angiography to exclude pulmonary embolus as a differential diagnosis. The source of sepsis included one case of primary native septic arthritis, one chronic tibial osteomyelitis and one bowel perforation. Two patients required surgical debridement to attempt to control their sepsis. Two patients died despite maximal organ support within 72 h of admission to critical care. All three patients had normal left ventricular systolic function, with low stroke volumes unresponsive to volume loading. Conclusions and implications Acute ventricular cardiomyopathy is a common complication of severe sepsis and is difficult to diagnose using standard invasive cardiac output devices commonly used within critical care areas. Trans thoracic echocardiography presents a point of care diagnostic modality that allows rapid, repeatable, reliable assessment of independent ventricular function. Low cardiac output states seen with acute dilated right ventricular cardiomyopathy appear to show little benefit from fluid boluses and poor response to standard pharmacological strategies for septic shock. In the two patients requiring invasive ventilation, death followed within 72 h of diagnosis of right ventricular cardiomyopathy. Surveillance scanning for a impaired and/or dilated right ventricle may allow earlier detection and exploration of alternative treatment strategies. Respiratory failure in severe septic shock associated with right ventricular cardiomyopathy appears to confer a significant increased mortality rate (66%) when compared with a global population (35%) and may aid in prognostication.

Value of intracardiac ultrasound in the diagnosis of right ventricular cardiomyopathy

Value of intracardiac ultrasound in the diagnosis of right ventricular cardiomyopathy

Value of intracardiac ultrasound in the diagnosis of right ventricular cardiomyopathy

Value of intracardiac ultrasound in the diagnosis of right ventricular cardiomyopathy

Complex arrhythmias in a patient with predominantly right ventricular cardiomyopathy

A 23-year-old man presented with ventricular tachycardia. The electrocardiogram revealed right bundle branch block plus right axis deviation. It also showed frequent episodes of asystole, 2:1 sinuatrial block and couplets of left bundle branch block morphology. Electrophysiologic study demonstrated sinuatrial entrance block, with an HV interval of 65 msec. It was also possible to induce sustained ventricular tachycardia of left bundle branch block pattern with normal QRS axis. Subsequent investigations were consistent with the diagnosis of right ventricular cardiomyopathy with mild left ventricular involvement.