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Isolated Right Ventricular Dilated Cardiomyopathy: An Early Diagnosis.

Briongos Figuero S, Acena Navarro A - J Clin Med Res (2015)

Bottom Line: A pulmonary hemodynamic study and a coronary angiography were strictly normal.Lastly pulmonary function tests and a pulmonary angiography were performed, which did not find any lung disease causing the right ventricular dilatation.The patient was catalogued as an early stage of an idiopathic form of right ventricular dilated cardiomyopathy.

View Article: PubMed Central - PubMed

Affiliation: Cardiology Department, Infanta Leonor Hospital, Madrid, Spain.

ABSTRACT
Because of an incomplete right bundle branch block, a severe right ventricular dilatation with no left ventricular cardiomyopathy was found in a 44-year-old man. Magnetic resonance and transesophageal echocardiography confirmed the finding and these tests also failed to find any potential cause. A pulmonary hemodynamic study and a coronary angiography were strictly normal. Lastly pulmonary function tests and a pulmonary angiography were performed, which did not find any lung disease causing the right ventricular dilatation. The patient was catalogued as an early stage of an idiopathic form of right ventricular dilated cardiomyopathy.

No MeSH data available.


Related in: MedlinePlus

Magnetic resonance, four-chamber view: diastolic (panel A) and systolic (panel B) frame, exhibiting normal right ventricular shortening. Transesophageal echocardiography, mid-esophageal plane (panel C) and also a color-Doppler (panel D) view exhibiting no interatrial shunt.
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Figure 2: Magnetic resonance, four-chamber view: diastolic (panel A) and systolic (panel B) frame, exhibiting normal right ventricular shortening. Transesophageal echocardiography, mid-esophageal plane (panel C) and also a color-Doppler (panel D) view exhibiting no interatrial shunt.

Mentions: A 44-year-old man was referred to the cardiology department because of an incomplete right bundle branch block (IRBBB) detected in the electrocardiogram (EKG). No epsilon or negative T waves were found on the EKG (Fig. 1). He had no history of familiar sudden cardiac death and he did not have any previous disease despite he was a carrier of C-hepatitis virus. No medication was needed for the treatment of that virus because transaminase enzymes in blood analysis were in normal range and abdominal echography showed normal liver morphology. He was only taking sertraline and bromazepam because of depressive syndrome. He remained completely asymptomatic, in NYHA functional class I/IV, and his physical examination did not show any abnormal finding. In order to begin the study of the IRBBB we first performed an echocardiogram in which a severe right ventricle (RV) dilatation (Fig. 1) and a moderate tricuspid regurgitation (TR) appeared. Left ventricle ejection fraction (LVEF) was preserved (55%) and mitral and aortic valves were normal, in function and morphology. The estimated systolic pulmonary pressure was normal and there was not any indirect sign of pulmonary hypertension. To better characterize the findings in the RV, a magnetic resonance (MR) was performed. It showed a diastolic diameter of the RV of 55 mm (Fig. 2) and confirmed the functional cause of the TR (ring dilatation). There were not any dyskinesia or akinesia areas (Supplementary Videos 1 and 2, www.jocmr.org) or pathologic gadolinium enhancement, and RV ejection fraction was 57%. Abnormal venous drainage was also excluded with the MR. The transesophageal echocardiography did not find any interatrial (Fig. 2) or interventricular septal defects. Holter monitoring did not find any arrhythmic event. Hemodynamic study showed normal pulmonary pressures (systolic pulmonary artery pressure/mean pulmonary artery pressure/diastolic pulmonary artery pressure of 24/14/7 mm Hg respectively) and normal pulmonary wedge pressure (12 mm Hg) which led us to exclude pulmonary hypertension as the cause of the RV dilatation. Cardiac ischemic disease was excluded by performing a coronary angiography. No atherosclerosis plaques in the coronary arteries were found. Pulmonary functions tests (forced expiratory volume in 1 s (FEV1): 117%; FEV1/forced vital capacity (FVC): 84%), diffusion capacity (DLCO: 115%), artery gasometry and polysomnographic study were also normal. We lastly performed a pulmonary angiography, in which a chronic thromboembolic pulmonary disease was also excluded. We decided not to start any medication due to the lack of symptoms of the patient and we also decided to keep a watchful waiting attitude. The patient was catalogued as an isolated form of right ventricular dilated cardiomyopathy due to the finding of an RV dilatation, without any potential cause for it. After 2 years of follow-up, the patient remains asymptomatic and the RV persists dilated.


Isolated Right Ventricular Dilated Cardiomyopathy: An Early Diagnosis.

Briongos Figuero S, Acena Navarro A - J Clin Med Res (2015)

Magnetic resonance, four-chamber view: diastolic (panel A) and systolic (panel B) frame, exhibiting normal right ventricular shortening. Transesophageal echocardiography, mid-esophageal plane (panel C) and also a color-Doppler (panel D) view exhibiting no interatrial shunt.
© Copyright Policy - open access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4554224&req=5

Figure 2: Magnetic resonance, four-chamber view: diastolic (panel A) and systolic (panel B) frame, exhibiting normal right ventricular shortening. Transesophageal echocardiography, mid-esophageal plane (panel C) and also a color-Doppler (panel D) view exhibiting no interatrial shunt.
Mentions: A 44-year-old man was referred to the cardiology department because of an incomplete right bundle branch block (IRBBB) detected in the electrocardiogram (EKG). No epsilon or negative T waves were found on the EKG (Fig. 1). He had no history of familiar sudden cardiac death and he did not have any previous disease despite he was a carrier of C-hepatitis virus. No medication was needed for the treatment of that virus because transaminase enzymes in blood analysis were in normal range and abdominal echography showed normal liver morphology. He was only taking sertraline and bromazepam because of depressive syndrome. He remained completely asymptomatic, in NYHA functional class I/IV, and his physical examination did not show any abnormal finding. In order to begin the study of the IRBBB we first performed an echocardiogram in which a severe right ventricle (RV) dilatation (Fig. 1) and a moderate tricuspid regurgitation (TR) appeared. Left ventricle ejection fraction (LVEF) was preserved (55%) and mitral and aortic valves were normal, in function and morphology. The estimated systolic pulmonary pressure was normal and there was not any indirect sign of pulmonary hypertension. To better characterize the findings in the RV, a magnetic resonance (MR) was performed. It showed a diastolic diameter of the RV of 55 mm (Fig. 2) and confirmed the functional cause of the TR (ring dilatation). There were not any dyskinesia or akinesia areas (Supplementary Videos 1 and 2, www.jocmr.org) or pathologic gadolinium enhancement, and RV ejection fraction was 57%. Abnormal venous drainage was also excluded with the MR. The transesophageal echocardiography did not find any interatrial (Fig. 2) or interventricular septal defects. Holter monitoring did not find any arrhythmic event. Hemodynamic study showed normal pulmonary pressures (systolic pulmonary artery pressure/mean pulmonary artery pressure/diastolic pulmonary artery pressure of 24/14/7 mm Hg respectively) and normal pulmonary wedge pressure (12 mm Hg) which led us to exclude pulmonary hypertension as the cause of the RV dilatation. Cardiac ischemic disease was excluded by performing a coronary angiography. No atherosclerosis plaques in the coronary arteries were found. Pulmonary functions tests (forced expiratory volume in 1 s (FEV1): 117%; FEV1/forced vital capacity (FVC): 84%), diffusion capacity (DLCO: 115%), artery gasometry and polysomnographic study were also normal. We lastly performed a pulmonary angiography, in which a chronic thromboembolic pulmonary disease was also excluded. We decided not to start any medication due to the lack of symptoms of the patient and we also decided to keep a watchful waiting attitude. The patient was catalogued as an isolated form of right ventricular dilated cardiomyopathy due to the finding of an RV dilatation, without any potential cause for it. After 2 years of follow-up, the patient remains asymptomatic and the RV persists dilated.

Bottom Line: A pulmonary hemodynamic study and a coronary angiography were strictly normal.Lastly pulmonary function tests and a pulmonary angiography were performed, which did not find any lung disease causing the right ventricular dilatation.The patient was catalogued as an early stage of an idiopathic form of right ventricular dilated cardiomyopathy.

View Article: PubMed Central - PubMed

Affiliation: Cardiology Department, Infanta Leonor Hospital, Madrid, Spain.

ABSTRACT
Because of an incomplete right bundle branch block, a severe right ventricular dilatation with no left ventricular cardiomyopathy was found in a 44-year-old man. Magnetic resonance and transesophageal echocardiography confirmed the finding and these tests also failed to find any potential cause. A pulmonary hemodynamic study and a coronary angiography were strictly normal. Lastly pulmonary function tests and a pulmonary angiography were performed, which did not find any lung disease causing the right ventricular dilatation. The patient was catalogued as an early stage of an idiopathic form of right ventricular dilated cardiomyopathy.

No MeSH data available.


Related in: MedlinePlus