A Case of Pulmonary Arterial Hypertension Improved with Normalization of a Prominent Negative Component of P Wave in Lead V1

S184 Journal of Cardiac Failure Vol. 18 No. 10S October 2012 he experienced nocturnal dyspnea and a routine annual health check revealed bilateral pleural effusion and bilateral hilar lymph adenopathy. He was diagnosed with congestive heart failure and visited our hospital on January 26th. He was admitted that day and 5 days later coronary angiography was performed. The results showed normal coronary arteries but the subsequent Ga scintigraphy revealed accumulation of Ga in the hilar lymph nodes bilaterally. The patient’s serum lysozyme level was found to be elevated and he was diagnosed with cardiac sarcoidosis by cardiac biopsy. We treated this patient with cardiac rehabilitation and steroids, ARB, and b blocker. His heart failure improved gradually and he was discharged on March 24th 2012. This patient reacted to treatment very favorably, with a quick improvement in his condition. The authors conclude that the combination of drugs and exercise therapy contributed to this improvement, as opposed to treatment with steroids alone.

severe pulmonary hypertension. The pulmonary capillary wedge pressure and the right atrial (RA) pressure were normal. Continuous intravenous epoprostenol therapy was initiated, resulting in improvement in mPAP (65mmHg) and CI (2.32L/min/m2). The normalization of TFP-V1 was accompanied by the improvement of clinical condition. In this patient, the chest CT performed around the same time as cardiac catheterization showed improvement in the RA dimensions at the level of the fourth intercostal space, decreasing from 34.8cm2 to 24.8cm2; therefore, it was suggested that TFP-V1 was reflected in RA enlargement. We similarly investigated 20 other pulmonary hypertension patients. There was a significant negative correlation between TFP-V1 and RA dimensions. Furthermore, TFP-V1 showed negative correlation with mPAP and CI. Generally, the negative component of P wave in lead V1 is an evidence of volume overload in left atrium; our study suggested that it is also found in pulmonary hypertension, indicating RA enlargement and severe pulmonary hypertension.

P-102 A Case of Female Danon Disease with Rapidly-deteriorated Heart Failure Heading for Heart Transplantation YUICHIRO IIDA, HISAHITO SHINAGAWA, TAKAYUKI INOMATA, TOYOJI KAIDA, TOSHIMI KOITABASHI, TOHRU IZUMI Department of Cardio-angiology, Kitasato University, School of Medicine, Kanagawa, Japan Background: Danon disease is an X-linked systemic disorder, characterized by cardiomyopathy, mental retardation and skeletal myopathy affecting young men. Female patients, however, are often diagnosed as idiopathic dilated cardiomyopathy (IDCM) because of the later-onset and isolated cardiac symptoms. Case Report: We report a 40-year-old female who was diagnosed as IDCM three years ago with severely-impaired left ventricular (LV) function. Cardiac magnetic resonance demonstrated marked late Gadolinium enhancement in extended LV wall and endomyocardial biopsy specimen from LV showed severe cardiomyocyte degeneration without intracellular vacuoles typical for lysosomal glycogen storage diseases. In spite of optical medical pharmacotherapy with cardiac resynchronization therapy, LV function has been rapidly deteriorated leading to end-stage HF dependent on intravenous inotropics, waiting for heart transplantation. Genetic testing her son affecting Danon disease provided a clue to her diagnosis through a nucleotide substitution in the lysosome-associated membrane protein 2 gene. Conclusion: Danon disease should be suspected in the middle-aged female manifesting as DCM having family members of male teenager with LV hypertrophy, because of its discouraged cardiac prognosis.

P-103 Angiosarcoma of the Pulmonary Artery Diagnosed by Delayed Enhancedment of MRI YOSUKE KAYAMA1, TAKUYA YOSHINO2, TATSUYA KOYAMA1, SHINGO SEKI1, MICHIHIRO YOSHIMURA2 1 Division of Cardiology, Internal Medicine, Katsushika Medical Center Jikei University School of Medicine, Tokyo, Japan, 2Division of Cardiology, Internal Medicine, Jikei University School of Medicine, Tokyo, Japan Primary pulmonary angiosarcoma is rare tumor, with fewer than 80 cases reported in Japan. The prognosis of subjects was very poor. Most patients die within 6 to 24 months of a diagnosis of pulmonary angiosarcoma. The benefits of the multimodality treatment such as chemotherapy and radiation therapy are unclear. Surgical resection remains the best treatment option at present. Therefore, it is important to accurately diagnose primary pulmonary angiosarcoma immediately using non-invasive modalities. Therefore, it is desirable that a means of early detection and diagnosis of such malignant tumors can be established. We present here a case of a Primary pulmonary angiosarcoma with right ventricular failure that was diagnosed by characteristic images of echocardiography and enhanced magnetic resonance image (MRI). This approach also enabled to differentiate between thrombus and malignant tumors before attempting surgery therapy. After resection, pathological examination revealed a intimal sarcoma of pulmonary artery. 10 months later, the patients died with progressive right ventricular heart failure in spite of several treatments for sarcoma.

P-104 A Case of Pulmonary Arterial Hypertension Improved with Normalization of a Prominent Negative Component of P Wave in Lead V1 HIROAKI OBATA, HIROMI KAYAMORI, MASATO ODA, TAKESHI KASHIMURA, HARUO HANAWA, MAKOTO KODAMA Division of Cardiology, Niigata University Graduate School of Medical and Dental Sciences, Niigata, Japan A 30-year-old man was hospitalized for progressive dyspnea and diagnosed with idiopathic pulmonary arterial hypertension. The electrocardiogram obtained at admission showed a prominent negative component of P wave in lead V1, and the P terminal force in lead V1 (TEP-V1) was -0.312mVsec. The mean pulmonary arterial pressure (mPAP574mmHg) and the cardiac index (CI51.74L/min/m2) revealed

P-105 A Rare Case with Fatal Pulmonary Tumor Thrombotic Microangiopathy YUTAKA MIURA, YOSHIHIRO FUKUMOTO, KOICHIRO SUGIMURA, KIMIO SATO, KOTARO NOCHIOKA, TATSUO AOKI, SHUNSUKE TATEBE, SAORI YAMAMOTO, HIROAKI SHIMOKAWA Department of Cardiovascular Medicine, Tohoku University Graduate School of Medicine, Sendai, Japan Case: 27 years-old, female. Clinical History: In April, 2011, she suffered from exertional dyspnea, and admitted to the hospital nearby. Echocardiography showed right ventricular dilatation and severe tricuspid valve regurgitation. Right heart catheterization showed elevated mean pulmonary arterial pressure (mPAP, 39 mmHg). After the diagnosis of pulmonary hypertension, her hemodynamic values were gradually deteriorated (blood pressure 100/60 mmHg with 5 mg/kg/min of dobutamine), and she was transferred to our hospital. We started her treatment with percutaneous cardiopulmonary support (PCPS), intravenous prostacycline and oral sildenafil; however, we were unable to obtain any hemodynamic improvement. Then, we added ambrisentan and imatinib, with which we were able to discontinue the 6-weeks PCPS treatment. As she had slight fever, bloody sputum, and high levels of tumor markers, we performed PET examination, which revealed the only mild uptake in the abdominal and mediastinal lymph nodes. We then performed abdominal echography and GI fiber examination, without any findings of malignancies. On September 20th, 2011, she passed away due to the respiratory failure, rapidly elevated mPAP, and hemodynamic disruption. Autopsy examination demonstrated the presence of multiple emboli of signet ring cells in the pulmonary small arteries, a compatible finding with pulmonary tumor thrombotic microangiopathy (PTTM). Conclusion: We experienced a rare case of fatal PTTM resistant to intensive care.

P-106 The Patient after CRT that Making a Graph of Body Weight Dramatically Reduced Hospitalization NAOKI NOZAKI Ayase Heart Hospital, Tokyo, Japan This case is 72 yrs old female. She was diagnosed as dileted cardiomyopathy in 1993 and she had been taking a medicine for chronic heart failure(CHF). Left ventricular systolic function was diffse severe hypokinesis (EF 33%) with dissynchrony. Although CRT-D implantation was performed in Dec. 2006, she had been hospitalized for worsening CHF over and over (4 times in 2007, 5 times in 2008, 1 time in 2009, 5 times in 2010, 3 times in 2011). Repeated guidance of restriction for salt and water intake was not effective. Whenever her hospitalization, her condition was easily improved by oxgen supply and using diuretics during 2 or 3 days. When she was discharged in April 2011, we tought her making a simple line graph of her own body weight every day. We also explained her appropriate body weight. She brings her body weight graph every consultation. Ever since, her condition has been stable and she has never been hospitalized for over 1 yr. Conclusion: In this case, not the record of body weight itself, but making line graph of own body weight significantly reduced her hospitalization.

P-107 Reversible Left Ventricular Dysfunction due to Hypothyroidism and Severe Obstructive Sleep Apnea; A Case Report TASUKU YAMAGUCHI, YOSHIFUMI TAKATA, YASUHIRO USUI, YOSUKE NISHIHATA, KOTA KATO, KAZUKI SHIINA, AKIRA YAMASHINA Department of Cardiology, Tokyo Medical University, Tokyo, Japan A 42 years old man was introduced to our hospital with suspicion of obstructive sleep apnea syndrome (OSAS). He was not obese but had a chief complaint of excessive daytime sleepiness. A polysomnography showed an apnea-hypopnea index (AHI) of 46.3/hr and an arousal index of 47.9/hr, he therefore was diagnosed as severe OSAS. The first laboratory examination showed high level of creatine kinase