Sometimes Surgery is the Only Way to Make a Diagnosis and Treat the Patient: A Case of Congenital Partial Absence of Pericardium.

INTRODUCTION: A 47-year-old Caucasian woman with a past medical history of multiple ablative procedures for supraventricular arrhythmias and pacemaker implantation presented with increasing shortness of breath. The initial working diagnosis of the team treating her was ablation-induced pulmonary stenosis, especially after the recording of increased flow velocities through the right lower pulmonary vein. CASE PRESENTATION: The patient was alert and oriented, but obviously dyspnoeic. The vital signs were normal. The physical examination revealed a soft cardiac systolic murmur and the lungs were clear on auscultation. The electrocardiogram showed a pacemaker rhythm. The echocardiogram showed borderline normal global systolic function of the left ventricle and severe mitral regurgitation. The transoesophageal echocardiogram confirmed the above findings and revealed increased velocities through the right lower pulmonary vein. The working diagnosis of ablation-induced pulmonary stenosis was reinforced by the cardiac CT angiography. The patient was subsequently referred for surgical intervention. The intra-operative findings were both unexpected and impressive: congenital partial absence of the pericardium was responsible for herniation of the right chambers into the pleural space. Mitral regurgitation was attributed to failure of coaptation due to the very short surface of the leaflets. Extensive external fibrosis around the pulmonary veins caused the pulmonary vein stenosis. CONCLUSION: The final diagnosis of a partial pericardial defect causing torsion and distortion of the heart chambers was made only at surgery. The consistent finding of pulmonary vein stenosis in the non-invasive modalities and the past medical history of ablations initially misleadingly led us to the assumption that they were related. LEARNING POINTS: The initial working diagnosis of ablation-induced pulmonary vein stenosis based on the patient's shortness of breath, severe mitral regurgitation, right lower pulmonary vein stenosis and past medical history of multiple ablative procedures, was incorrect.The finding that the cardiac silhouette was borderline displaced to the right, the mildly hypoplastic right lung and the borderline impairment of the global systolic function of the left ventricle, especially after coronary artery disease was ruled out, should have been given greater consideration.Cardiac computer tomographic images taken 7 years ago were used to assess the severity of the right inferior pulmonary vein stenosis; new medical technologies will provide even better diagnostic techniques.

Both ramipril and telmisartan reverse indices of early diabetic cardiomyopathy: a comparative study.

AIMS: We tested the hypothesis that renin-angiotensin system inhibition could reverse left ventricular diastolic dysfunction in patients with type 2 diabetes. METHODS AND RESULTS: Forty asymptomatic patients with type 2 diabetes were recruited in this double-blind cross-over trial. Left ventricular diastolic function was assessed at baseline with Doppler echocardiography; ratios of early to late peak flow velocity through the mitral orifice (E/A) and velocity time integral of early to late transmitral diastolic flow (VTIE/VTIA) were evaluated. In addition, plasma brain natriuretic peptide (BNP) was measured. Patients received randomly either ramipril (2.5 mg/day), or telmisartan (40 mg/day) or their combination for 3 months. Subsequently, every patient was crossed over to alternative regimens after a 2-week washout period. Measurements were repeated at the end of each treatment period. Both E/A and VTIE/VTIA ratios were increased (29 and 20% with ramipril, 25 and 23% with telmisartan and 36 and 28% with combination treatment, respectively, p < 0.001), whereas plasma BNP levels were significantly reduced with all 3 regimens (9% with ramipril, 25% with telmisartan and 36% with combination, p < 0.001). CONCLUSIONS: Both ramipril and telmisartan improve echocardiographic left ventricular diastolic indices and reduce plasma BNP levels in diabetic patients; their combination yields an even better therapeutic effect.

Comparison of the effects of ramipril versus telmisartan in reducing serum levels of high-sensitivity C-reactive protein and oxidized low-density lipoprotein cholesterol in patients with type 2 diabetes mellitus.

The effect of ramipril (an angiotensin [AT]-converting enzyme inhibitor), telmisartan (an AT-II type 1 receptor blocker), or their combination on inflammation and lipid peroxidation was assessed in 37 patients with type 2 diabetes who were free of coronary artery disease. All regimens were associated with a significant reduction of C-reactive protein and oxidized low-density lipoprotein cholesterol serum levels (p <0.001). These results further enlighten the mechanisms underlying the cardiovascular beneficial effect of renin-AT system inhibition.