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. .

Dual-Chamber pacing for postoperative residual left ventricular gradient after aortic valve replacement and concomitant septal myectomy for severe aortic stenosis and subvalvular obstruction.

BACKGROUND: Aortic stenosis is occasionally associated with subvalvular obstruction and remaining obstruction may at times be found after aortic valve replacement. CASE REPORT: A 69-year-old woman with a history of several episodes of unconsciousness was admitted because of exertional chest oppression. The echocardiography revealed severe aortic stenosis (flow velocity 6.2 m/sec, maximum / mean pressure gradient 152 / 99 mmHg, valve area 0.59 cm2) as well as gradient within the left ventricular cavity from the mid ventricular level (flow velocity 4.5 m/sec, maximum gradient 82 mmHg). Despite aortic valve replacement and concomitant septal myectomy which was thought adequate in reducing subvalvular pressure gradient during surgery, postoperative echocardiography revealed significant residual mid ventricular gradient (flow velocity 4.9 m/sec, maximum gradient 95 mmHg). It was decided to implant dual-chamber pacemaker, which resulted in significant reduction of residual mid ventricular gradient (flow velocity 1.4 m/sec, maximum gradient 8 mmHg). CONCLUSION: Dual-chamber pacing was extremely effective in reducing residual mid ventricular gradient in a patient who underwent aortic valve replacement and concomitant septal myectomy for severe aortic stenosis and subvalvular obstruction..

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.

Six underlying health conditions strongly influence mortality based on pneumonia severity in an ageing population of Japan: a prospective cohort study.

BACKGROUND: Mortality prediction of pneumonia by severity scores in patients with multiple underlying health conditions has not fully been investigated. This prospective cohort study is to identify mortality-associated underlying health conditions and to analyse their influence on severity-based pneumonia mortality prediction. METHODS: Adult patients with community-acquired pneumonia or healthcare-associated pneumonia (HCAP) who visited four community hospitals between September 2011 and January 2013 were enrolled. Candidate underlying health conditions, including demographic and clinical characteristics, were incorporated into the logistic regression models, along with CURB (confusion, elevated urea nitrogen, tachypnoea, and hypotension) score as a measure of disease severity. The areas under the receiver operating characteristic curves (AUROC) of the predictive index based on significant underlying health conditions was compared to that of CURB65 (CURB and age ≥ 65) score or Pneumonia severity index (PSI). Mortality association between disease severity and the number of underlying health conditions was analysed. RESULTS: In total 1772 patients were eligible for analysis, of which 140 (7.9%) died within 30 days. Six underlying health conditions were independently associated: home care (adjusted odds ratio, 5.84; 95% confidence interval, CI, 2.28-14.99), recent hospitalization (2.21; 1.36-3.60), age ≥ 85 years (2.15; 1.08-4.28), low body mass index (1.99, 1.25-3.16), neoplastic disease (1.82; 1.17-2.85), and male gender (1.78; 1.16-2.75). The predictive index based on these conditions alone had a significantly or marginally higher AUROC than that based on CURB65 score (0.78 vs 0.66, p = 0.02) or PSI (0.78 vs 0.71, p = 0.05), respectively. Compared to this index, the AUROC of the total score consisting of six underlying health conditions and CURB score (range 0-10) did not improve mortality predictions (p = 0.3). In patients with one or less underlying health conditions, the mortality was discretely associated with severe pneumonia (CURB65 ≥ 3) (risk ratio: 7.24, 95%CI: 3.08-25.13), whereas in patients with 2 or more underlying health conditions, the mortality association with severe pneumonia was not detected (risk ratio: 1.53, 95% CI: 0.94-2.50). CONCLUSIONS: Mortality prediction based on pneumonia severity scores is highly influenced by the accumulating number of underlying health conditions in an ageing society. The validation using a different cohort is necessary to generalise the conclusion.

Comprehensive arrhythmic surgery for sustained ventricular tachycardia revisited.

A 68-year-old woman with a history of old inferior myocardial infarction was admitted because of sustained ventricular tachycardia. Double vessel coronary disease was found with subtotal obstruction of the right coronary artery (#1) and severe stenosis of the left circumflex coronary artery (#13). Dilated left ventricle with a large ventricular aneurysm at the inferior wall and severely reduced systolic function were also present. A comprehensive surgical ablation using subendocardial resection and cryoablation was performed in combination with aneurysmectomy, left ventricular reconstruction, and coronary bypass grafting to the circumflex coronary artery. An implantable cardioverter-defibrillator was also implanted. She has remained well without recurrence of sustained ventricular tachycardia for one year. Although radiofrequency catheter ablation is nowadays considered a first-line therapy for patients with sustained ventricular tachycardia, surgical ablation remains a valuable option for selected patients, particularly for those requiring left ventricular aneurysmectomy and coronary artery bypass grafting, in order to gain a better long-term prognosis through total cure of ventricular tachycardia. .

Subacute effusive-constrictive pericarditis: Echocardiography-guided diagnosis and management.

A 49-year-old man presented with flu-like symptoms of two weeks. Electrocardiogram showed diffuse ST elevation. Blood samples revealed severe renal failure and moderate inflammatory results. Echocardiogram showed large pericardial effusion, dilated inferior vena cava, but no right ventricular collapse. The patient underwent hemodialysis, after which he developed clinical signs of cardiac tamponade with echocardiographic features of collapse of the right ventricle. Pericardial drainage was then performed revealing purulent fluid of 800 ml. Streptococcus agalactiae was found in the cultures of urine, blood, and pericardial fluid. Despite removal of the pericardial fluid, echocardiogram failed to show any improvement in dilated inferior vena cava and estimated right atrial pressure remained elevated. Thus, a diagnosis of subacute effusive-constrictive pericarditis was made. Following antibiotic treatment for purulent pericarditis, early pericardiectomy was performed under transesophageal echocardiographic monitoring which successfully guided surgeons to careful removal of thick and adhesive visceral pericardium as well as an additional Waffle procedure resulting in significant clinical and hemodynamic improvement. Echo-guided approach is most practical in establishing the diagnosis of effusive-constrictive pericarditis and also most helpful in obtaining successful surgical results. .

Late rupture of artificial neochordae associated with hemolytic anemia.

A 63-year-old man, status post-mitral valve repair for severe mitral regurgitation secondary to ruptured chordae of the anterior leaflet, was admitted seven years after surgery because of a recent history of hematuria. A new apical pansystolic murmur was audible. Hemoglobin level was 5.7 g/dL. Results of other hematologic studies and a peripheral blood smear were indicative of mechanical hemolysis. Transesophageal echocardiography showed a high-velocity jet of mitral regurgitation that directly collided with the annuloplasty ring. At re-operation, one of the artificial neochordae to A2 and A3 segments was found to be disrupted. The mitral valve was replaced with a 33/31 mm On-X valve. Hemolytic anemia disappeared immediately after surgery. Although mitral valve repair with artificial neochodae has been shown to have long-term durability, it should be recognized that artificial neochordae may rupture a long time after mitral valve repair. Also, although hemolytic anemia is known as an early complication after mitral valve repair, it is worth knowing that hemolytic anemia may occur as a late complication after mitral valve repair. Continuous long-term monitoring of the patients after mitral valve repair is recommended. .

Risk factor profiles of stroke, myocardial infarction, and atrial fibrillation: a Japanese Multicenter Cooperative Registry.

OBJECTIVE: We sought to clarify risk factor profiles and current treatment of Japanese patients with stroke, myocardial infarction (MI), and nonvalvular atrial fibrillation (NVAF) using the database of the Japan Thrombosis Registry for Atrial Fibrillation, Coronary, or Cerebrovascular Events (J-TRACE). METHODS: J-TRACE is a nationwide multicenter cooperative cohort of Japanese patients with MI, stroke, and NVAF. Baseline characteristics of 8087 Japanese patients (5804 male, average age 68.7 years) with history of stroke (n=3554), MI (n=2291), or NVAF (n=2242) were analyzed. RESULTS: History of stroke (14.7%) was more frequent than history of MI (2.6%) in patients with stroke, whereas history of stroke (6.6%) was less frequent than history of MI (7.6%) in patients with MI. In patients with NVAF, history of stroke (14.3%) was far more frequent than history of MI (3.4%). Hypertension was more frequent in stroke (74.4%) than MI (62.0%) or NVAF (57.7%), whereas hypercholesterolemia, diabetes mellitus, and cigarette smoking were more prevalent in patients with MI (56.1%, 35.1%, and 33.3%, respectively) than in those with stroke (35.7%, 22.4%, and 19.7%, respectively) or NVAF (26.9%, 17.2%, and 16.1%, respectively). Alcohol consumption (34.9%) and obesity (body mass index>25) (32.8%) were most common in patients with NVAF. In all patients, nonmedication rates were higher in patients with hypercholesterolemia (29.8%) or diabetes (36.9%) than in those with hypertension (9.5%). Warfarin was used in 58.9% of patients with low-risk and 75.4% with high-risk NVAF. CONCLUSION: Risk factor profiles and their modification were not similar among patients in Japan with MI, stroke, and NVAF, although they share a high risk of thrombotic events.