Pulmonary hypertension secondary to interstitial fibrosis with pulmonary venous lesions masquerading pulmonary veno-occlusive disease.

We present a 41-year-old man with idiopathic interstitial pneumonia and pulmonary hypertension (PH) in the setting of a non-autoimmune background whose clinical presentation masqueraded pulmonary veno-occlusive disease (PVOD). Because of no histological evidence of venous occlusion in his previous lung biopsy, phosphodiesterase type-5 inhibitor was given, resulting in sudden onset of pulmonary edema. At autopsy, there were histological features of interstitial fibrosis with occlusion of the lobular septal veins and venules. Clinical presentations of PH due to interstitial fibrosis with pulmonary venous lesions may simulate those of PVOD and careful diagnostic and therapeutic approaches are required.

Intracardiac Thrombosis and Systemic Embolism in a Patient with Cardiac Amyloidosis in Sinus Rhythm.

Intracardiac thrombosis formation in patients in sinus rhythm is a rare phenomenon. An 84-year-old woman was admitted because of worsening dyspnea on exertion. An electrocardiogram showed sinus rhythm, left atrial overload, marked left axis deviation, low voltage, and poor r-wave progression in leads V1-4. An echocardiogram showed relatively preserved left ventricular ejection fraction with minimal wall thickening. Her serum level of B-type natriuretic peptide (931 pg/mL) was markedly elevated and a diagnosis of worsening heart failure was made. During the course of treatment for heart failure, she was complicated by acute abdominal aortic thromboembolism together with left atrial thrombus. An emergency abdominal aortic thrombectomy was followed by the removal of a left atrial thrombus 2 days later. Left ventricular biopsy performed during the surgery revealed amyloid deposits in the myocardial interstitium. Immunohistochemical study confirmed the diagnosis of transthyretin cardiac amyloidosis. It is postulated that the risk of intracardiac thrombosis and systemic embolism is increased even in sinus rhythm in patients with cardiac amyloidosis.

Fulminant lymphocytic myocarditis: Prediction of successful weaning from Impella support for cardiogenic shock.

We present a series of four patients with biopsy-proven fulminant lymphocytic myocarditis with cardiogenic shock and discuss whether it is possible to predict recovery of left ventricular function and successful weaning at the time of initial placement of mechanical circulatory support. Impella CP (Abiomed, Danvers, MA, USA) was placed in these patients on admission. Patients 1 and 2 made complete recovery. Patient 3 proceeded to bi-ventricular assist device and is currently waiting for transplantation. Patient 4 proceeded to Impella 5 but died from multiple organ failure. Although the Impella provides excellent hemodynamic support, outcomes of the patients with fulminant myocarditis with Impella support may depend upon the severity of myocarditis and myocardial failure. In addition to the previously reported predictors such as the level of elevated biomarkers, the severity of ventricular wall edema, and the development of rhythm disturbances, the absence of right ventricular dysfunction seems important to predict successful weaning from Impella support. .

Primary Cardiac Lymphoma: A Lesson Learned from an Unsuccessful Experience.

A 79-year-old man was admitted because of complete heart block. Echocardiograms showed an abnormal mass adjacent to the sinus of Valsalva. Subsequent surgical resection was not successful. Despite chemotherapy, the patient died from multiple organ failure. It is important to recognize that approximately 80% of cases of cardiac lymphoma are diffuse large B-cell lymphoma, which is the only malignant neoplasm that may respond well to chemotherapy with rituximab. In order to save patients' lives, the early implementation of chemotherapy with rituximab is critical and should be considered as a therapeutic diagnostic option in select patients.

Comparison of Long-Term Mortality in Patients With Previous Coronary Artery Bypass Grafting Who Underwent Percutaneous Coronary Intervention With Versus Without Optimal Medical Therapy.

Although current guidelines have highlighted the importance of evidence-based optimal medical therapy (OMT) in patients with previous coronary artery bypass grafting (CABG), the effect of OMT on post-CABG patients requiring secondary coronary revascularization on prognosis remains unknown. We sought to examine the impact of OMT on post-CABG patients who underwent percutaneous coronary intervention (PCI) as secondary revascularization. A total of 632 consecutive post-CABG patients who underwent PCI between 2001 and 2013 at our hospital (84% men, median age 71 years) were divided into 2 groups: patients who were discharged with OMT and patients who were discharged without OMT (non-OMT). OMT was defined as the combination of an antiplatelet agent, statin, ß blocker, and angiotensin-converting enzyme inhibitor or angiotensin receptor blocker. Despite having a higher prevalence of clinical comorbidities, patients with OMT (n = 163) had a lower prevalence of all-cause death than those without OMT (n = 469) during a median follow-up of 95 months (OMT group 21.5%, non-OMT group 34.1%, p = 0.002). Both groups had similar procedural success rates. In a propensity-matched cohort (n = 146 each), OMT was associated with lower rates of all-cause death and cardiac death than non-OMT 8 years after PCI. In multivariable analysis, OMT was an independent predictor of all-cause death (hazard ratio [HR] 0.49, 95% confidence interval [CI] 0.34 to 0.72, p <0.001). In conclusion, OMT plays a protective role and reduces all-cause death in post-CABG patients requiring subsequent PCI. Outside of the domain of coronary revascularization, OMT could be considered an essential treatment in this patient population.