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..

Usefulness of Screening for Abdominal Aortic Aneurysm During Transthoracic Echocardiography in Women ≥50 Years of Age.

Usefulness of screening for abdominal aortic aneurysm (AAA) during transthoracic echocardiography (TTE) in women is uncertain. The aim of the present study was to clarify the clinical usefulness of screening for AAA during TTE and to identify important TTE indices associated with AAA in women in a routine clinical setting. We prospectively studied 1,495 women (≥50 years) referred for TTE. AAA was defined as ≥30 mm in size. The additional screening time for AAA was <1 minute. The abdominal aorta was visualized in 95.1 % (1,422 of 1,495) using the same TTE probe. AAA was identified in 1.9% (27 of 1422). The aortic root size was larger in patients with AAA than those without (33.3 ± 3.2 vs 30.5 ± 3.4 mm, p < 0.001). The aortic root size had a correlation with abdominal aortic size (r = 0.22, p < 0.001). The aortic root size of ≥30.3 mm was predictive of AAA (area under the curve = 0.74, p < 0.001) and all patients with AAA had the aortic root size of ≥28.0 mm. Multiple logistic regression analysis revealed that the aortic root size (Odds ratio 1.17, p = 0.007) was a most independent TTE index of AAA. In conclusion, the visibility of the abdominal aorta using TTE probe was excellent. When the aortic root size is ≥28.0 mm during TTE in women ≥50 years of age, screening for AAA should be carried out.

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.

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. .

Epstein-Barr virus-infected subcutaneous panniculitis-like T-cell lymphoma associated with methotrexate treatment.

Methotrexate-associated lymphoproliferative disorder (MTX-LPD) is a lymphoid proliferation or lymphoma in patients treated with MTX. We report a case of Epstein-Barr virus (EBV)-positive subcutaneous panniculitis-like T-cell lymphoma (SPTCL) in a 60-year-old Japanese man treated with MTX for rheumatoid arthritis (RA). SPTCL is a rare cytotoxic T-cell lymphoma characterized by involvement of subcutaneous fat mimicking panniculitis. The patient, who had been on MTX therapy for RA, manifested high fever and lumbago. Physical examination showed multiple subcutaneous nodules on the trunk including axillary and inguinal regions. Biopsy of the inguinal nodule showed profuse infiltration of CD8(+) T-cell lymphoma cells in the subcutaneous adipose tissues. A diagnosis of SPTCL was made according to the diagnostic criteria of World Health Organization classification. EBV-encoded small RNA in situ hybridization revealed that the lymphoma cells contained EBV genome. The cells were positive for EBV latent membrane protein 1, but not for EBNA2. After discontinuation of MTX, the nodules regressed spontaneously. Studies have reported that most MTX-LPDs are B-cell type lymphomas and Hodgkin lymphoma. To the best of our knowledge, EBV-positive SPTCL has not been reported in patients receiving MTX. Our case emphasizes the importance of clinical and virological characterization of MTX-associated SPTCL.