Biphasic pulmonary blastoma with rapid progression

We report a rare case of biphasic pulmonary blastoma (BPB) with rapid progression in a 75-year-old man. Computed tomography (CT) of the chest revealed a well-defined tumor shadow measuring 8 × 6 cm in the right upper lobe. CT-guided lung biopsy revealed malignant cells with sarcomatous elements. Right upper lobectomy and systemic lymph node dissection with chest wall resection were performed. Histopathologically, the tumor was composed of immature embryoniclike mesenchymal and epithelial components that resembled embryonic lung tissue. The final diagnosis was BPB classified as pathological stage IIB (T3N0M0). Two months later, the tumor recurred in the right supraclavicular lymph nodes. The patient died of respiratory failure due to tumor progression 7 months after surgery.     

Emergency off-pump complete arterial revascularization in a patient with dextrocardia

We describe complete emergency arterial coronary artery bypass grafting performed on the beating heart of a 73-year-old man with situs inversus totalis and triple-vessel disease. The right internal mammary artery was anastomosed to the left anterior descending artery in situ. The first and second obtuse marginal branches of the circumflex coronary and the posterior descending branch of the right coronary artery were sequentially revascularized using the left internal mammary and radial arteries in situ. The only abnormality was that the position of the heart mirrored that of a normal heart. Beating heart surgery appears to be as safe in patients with dextrocardia as in the general population. However, the position of the surgeon must be reconsidered for optimal handling of stabilizers and to facilitate access to anastomosis sites. Understanding mirror-image coronary arterial anatomy is important for successful surgical outcomes among patients with dextrocardia.     

Bilateral multiple sclerosing hemangiomas of the lung

This report presents a case of bilateral multiple sclerosing hemangiomas of the lung in a 73-year-old woman. A computed tomography (CT) scan of the chest showed a total of three well-defined small nodules in the right and left lower lobes of the lung. Because malignant metastatic lung tumors were highly suspected, a wedge resection of the left lower lobe was performed to obtain a definitive diagnosis. Histopathologically, two tumors of the left lower lobe were composed of epithelial-like cuboidal cells covering the surface of papillary protrusions and sheets of round to polygonal cells underneath the epithelial-like cells. The final diagnosis was that both tumors were sclerosing hemangiomas. After surgery the residual lesion of the right lower lobe was carefully followed by chest CT. The size of the right lung nodule did not change over the course of 9 years, and no new lesion has emerged.     

Differences in skin sympathetic involvements between two chronic autonomic disorders: multiple system atrophy and pure autonomic failure

ObjectiveCertain stimuli evoke increased sweat secretion (sympathetic sweat response; SSwR) and reduced skin blood flow (skin vasomotor reflex; SkVR) in the palm/sole. We evaluated SSwR and SkVR in patients with multiple system atrophy (MSA) and pure autonomic failure (PAF).MethodsSSwR and SkVR on the palm in response to deep inspiration and mental arithmetic were recorded in 11 MSA patients, 11 PAF patients, and 11 healthy controls. In addition, the head-up tilt test was performed, and the coefficient of variation of R–R intervals (CV_R–R) was obtained.ResultsSSwR amplitudes were significantly lower in the MSA and PAF patients than the controls. SkVR amplitudes in the PAF patients were significantly lower than the controls, but preserved in the MSA patients. In head-up tilt tests, all MSA and PAF patients showed orthostatic hypotension, with similar severity. CV_R–R was low in the MSA and PAF patients, but a significant difference was found only between the PAF and control groups.ConclusionIn the MSA patients, SkVR was preserved, but SSwR was diminished. In the PAF patients, both SkVR and SSwR were attenuated. The combination of SkVR and SSwR tests may differentiate MSA and PAF.     

Investigation of major genetic polymorphisms in the Renin-Angiotensin-aldosterone system in subjects with young-onset hypertension selected by a targeted-screening system at university

Although polymorphisms in renin-angiotensin-aldosterone (RAA) system genes for angiotensinogen (AGT M235T), angiotensin-converting enzyme (ACE I/D), angiotensin II type 1 receptor (AT1 A/C1166), and aldosterone synthase (CYP11B2-344T/C) have been major targets for genetic investigation in association with essential hypertension (EH), the influence of these genetic factors is still to be determined. Because patients with young-onset EH are thought to possess a stronger genetic background than EH patients who show elevated BP relatively late in life, the targeted screening of hypertensive students in Tohoku University was completed for the selection of subjects for genetic investigation. Out of 16,434 students (12,794 males and 3,670 females) younger than 30, 22 students showed a high blood pressure (BP) (systolic and diastolic BP of 140 and/or 90 mmHg or greater, respectively, on two occasions and more than 135 and/or 85 mmHg, respectively, at a third measurement during casual BP measurements at the Tohoku University Health Center. These 22 students were asked to measure their BP at home (HBP). Six of the students had a systolic HBP of more than 135 mmHg and/or a diastolic HBP of more than 85 mmHg, and these students subsequently received medical examinations at Tohoku University Hospital and were diagnosed with EH. Genotyping for the four major genetic polymorphisms mentioned above was performed on the six students with EH and on 12 of the remaining 16 students whose HBP was within the normal range (white coat hypertension: WCH). Neither the EH nor the WCH students showed a different distribution of genotypes and allelic frequencies, compared to those found in the general Japanese population. Hence, the present study suggests that none of the major genetic polymorphisms in the RAA system strongly influence the onset of EH.     

Massive pulmonary thromboembolism in pregnancy rescued using transcatheter thrombectomy

We report a rescued 37-year-old woman in her 30(th) week of gestation with massive pulmonary thromboembolism who was admitted to our cardiac care unit with progressive dyspnea and 2 episodes of syncope. Helical chest CT showed massive pulmonary thromboembolism of both pulmonary arteries. Although 26,000 U/day of heparin was administered following insertion of a temporary filter, hemodynamic evaluation documented no improvement. Since pulmonary artery (PA) pressure increased from 62/22 mmHg to 80/24 mmHg just after an emergency cesarean section on day 2, an emergency transcatheter thrombectomy was performed and it showed decreased PA pressure following extensive thrombus aspiration. Mother and baby were discharged with no complications.     

Gradual improvement of liver function after administration of ursodeoxycholic acid in an infant with a novel ABCB11 gene mutation with phenotypic continuum between BRIC2 and PFIC2

OBJECT: The authors report the case of a boy with PFIC type 2 or BRIC type 2 who suffered from liver dysfunction at 2 months after birth. METHODS AND RESULTS: A liver biopsy specimen revealed mild liver cirrhosis, and the findings resembled those observed in Byler disease. Genetic examination revealed a normal familial intrahepatic cholestasis-1 gene, but a heterozygous mutation for the ABCB11, C1620A (F540L), was observed. Therefore, the patient was initially diagnosed with PFIC type 2. For 3 years after the diagnosis, he had severe pruritus, an increased serum bile acid, and normal serum values of gamma-glutamyl transaminase. At the age of 2, treatment with administration of ursodeoxycholic acid was started; subsequently, a gradual improvement in his liver function was observed. At the age of 3, he suffered from massive intestinal and pulmonary hemorrhage, which improved immediately after the administration of vitamin K. He was then admitted to our hospital for liver transplantation. At 1 month after the admission, his liver dysfunction showed further improvement, except for a mild increase in the serum bile acid level. This condition did not show any change during the 5-year follow-up period. In addition, the patient showed severe growth failure and was diagnosed with growth hormone deficiency. Hence, he receives growth hormone administration. CONCLUSION: The patient could be genetically diagnosed with bile salt export pump disease of PFIC type 2 or BRIC type 2. Various clinical features are observed in PFIC or BRIC patients with ABCB11 mutation.     

Mitral annuloplasty as a ventricular restoration method for the failing left ventricle: a pilot study

BACKGROUND AND AIM OF THE STUDY: Undersized mitral annuloplasty (MAP) is effective in patients with dilated cardiomyopathy and functional mitral regurgitation (MR) since, as well as addressing the MR, the MAP may also reshape the dilated left ventricular (LV) base. However, the direct benefits of this possible reshaping on LV function in the absence of underlying MR remain incompletely understood. The study aim was to identify these benefits in a canine model of acute heart failure. METHODS: Six dogs underwent MAP with a prosthetic band on the posterior mitral annulus, using four mattress sutures. The sutures were passed individually through four tourniquets and exteriorized untied via the left atriotomy. Sonomicrometry crystals were implanted around the mitral annulus and left ventricle to measure geometry and regional function. Acute heart failure was induced by propranolol and volume loading after weaning from cardiopulmonary bypass; an absence of MR was confirmed by echocardiography. MAP was accomplished by cinching the tourniquets. Data were acquired at baseline, after induction of acute heart failure, and after MAP. RESULTS: MAP decreased mitral annular dimensions in both commissure-commissure and septal-lateral directions. Concomitantly, the diastolic diameter of the LV base and LV sphericity decreased (i.e., improved) from 37.4 +/- 9.3 to 35.9 +/- 10 mm (p = 0.063), and from 67.9 +/- 18.6% to 65.3 +/- 18.9% (p = 0.016), respectively. Decreases were evident in both LV end-diastolic pressure (from 17 +/- 7 to 15 +/- 6 mmHg, p = 0.0480 and Tau (from 48 +/- 8 to 45 +/- 8 ms, p <0.01), while fractional shortening at the LV base increased from 7.7 +/- 4.5% to 9.4 +/- 4.5% (p = 0.045). After MAP, increases were identified in both cardiac output (from 1.54 +/- 0.57 to 1.65 +/- 0.57 1/min) and Emax (from 1.86 +/- 0.9 to 2.41 +/- 1.31 mmHg/ml). CONCLUSION: The data acquired suggest that isolated MAP may have certain benefits on LV dimension/function in acute heart failure, even in the absence of MR. However, further investigations are warranted in a model of chronic heart failure.