PP-079 CONGENITAL ABSENCE OF THE LEFT CIRCUMFLEX CORONARY ARTERY

S64

Cardiology Posters / International Journal of Cardiology 140, Supplement 1 (2010) S1–S93

PP-076 TYPE B AORTIC DISSECTION AFTER BENTALL SURGERY IN A MARFAN PATIENT

PP-078 VENTRICULAR NONCOMPACTION IN CHILDREN: CLINICAL CHARACTERISTICS AND COURSE

Ibrahim Ece, Feyza Aysenur Pac, Mustafa Pac, Seref Alp Kucuker, Mehmet Burhan Oflaz, Sevket Balli, Esin Kibar Turkiye Yuksek Ihtisas Hastanesi, Ankara, Turkey

Senem Ozgur, Filiz Senocak, Utku Arman Orun, Burhan Ocal, Selmin Karademir, Vehbi Dogan, Osman Yilmaz Sami Ulus Children’s Hospital, Ankara, Turkey

Objective: Marfan syndrome is a dominantly inherited connective tissue disease characterized by cardiovascular, skeletal and ocular manifestations. The progressive dilatation of the proximal aorta leading to dissection and rupture is the typical feature of the disease. Aortic aneurysm and aortic rupture are the lethal complications of the disorder. Distal aortic disease may evolve in Marfan patients after aortic root surgery. In this report, we presented a patient with Marfan syndrome with previous Bentall operation for type-A aortic dissection, rapidly development type-B aortic dissection. Methods: The patient was operated 3 years ago for type-A aortic dissection. After aortic root surgery, annual mean growth rate of descending aorta was detected 5 mm. Due to rapid growth of the descending aorta diameter, elective surgical treatment was planned. The patient was admitted with chest pain before the operation time. The peak gradient assessed by echocardiography across the narrowing at the beginning part of the aneurysm was measured 62 mmHg and largest diameter of the aneurysm was 3.9 cm. The diagnosis of acute type-B aortic dissection was thought by echocardiography and confirmed by computed tomography. Results: The patient was operated. Follow-up 1 year postoperatively showed the patient was doing well clinically and hemodynamically. Conclusions: We suggest that all Marfan patients after aortic root surgery should undergo regular echocardiography to detect the progression of distal aortic disease at an early stage. Marfan syndrome and annual growth rate of descending aortic diameter more than 5 mm were predictors for retreatment. These patients require very close follow up by echocardiography and a plan for retreatment on the descending aorta to prevent sudden rupture late death.

Objective: Ventricular non compaction (VNC) is a rare cardiomyopathy characterized by prominent trabeculations and deep intratrabecular recesses which are in direct contact with intraventricular cavity. Its clinical manifestations include severe left ventricular dysfunction, arrhythmias, systemic embolism and sudden death. In this study, we aimed to identify the clinical characteristics of children with ventricular non compaction, evaluate the clinical course and determine the factors affecting prognosis. Methods: We retrospectively evaluated 29 children with VNC followed at Dr.Sami Ulus Children’s Hospital Pediatric Cardiology Department from December 2004 to November 2009. All patients’ age, gender, clinical presentations, symptoms at the time of diagnosis, associated cardiac and extracardiac abnormalities were also recorded.Echocardiographic examination was performed in all patients and 24 hours Holter examination was done in case of suspected arrhythmia. Results: Our ventricular noncompaction patients were 11.3% of all patients that we follow up because of different types of cardiomyopathies. There were 13 girls (44.8%) and 16 boys (55.2%) and mean age at presentation was 4.8±4.6 year. (1 month - 15 years). The mean duration of follow-up was 1.3±1.1 years (2 month - 4 years).At the diagnosis, 26 patients (89.6%) had left ventricular systolic dysfunction. Eight children (27.5%) had associated cardiac lesions and another 8 patients (27.5%) had extracardiac problems Tachypnea, failure to thrive, reccurrent pneumonia and fatigue were most commonly seen presenting symptoms. Two patients (6.8%) were asymptomatic at the time of diagnosis. Different arrhythmias were detected in 27.5% of the cases. Six patients (20.6%) died on follow up. Although there was no statistical significance; early presentation age and high left ventricular end diastolic diameter (LVEDD) at the diagnosis associated slightly with poor prognosis. Conclusions: Ventricular noncompaction patients consisted 11.3% of our all cardiomyopathic cases. Of all the patients 75.8% had isolated VNC, the other 24.2% had different associated heart anomalies, especially septation defects. In 27.5% of cases extracardiac diseases, mainly mental motor retardation were found. Interestingly, none of them was suffering from a neuromuscular disease which were reported to be frequent with VNC. The most common complaints at admission were due to heart failure (44.8%). The mortality rate was 20.6% and the reasons of death were cardiac failure and sepsis. No prognostic factor was found to be statistically important.

PP-077 NONSUSTAINED POLIMORPHIC VENTRICULER TACHYCARDIA WITH CORONARY ARTERY ANOMALY; LAD ORIGINATING FROM RCA OSTIUM Banu Evranos, Sercan Okutucu, Hakan Aksoy, Hikmet Yorgun, Kudret Aytemir, Lale Tokgozoglu, Hilmi Ozkutlu, Ali Oto Cardiology Department, Hacettepe University, Ankara, Turkey Objective: Coronary artery anomalies are common, occurring in 1-2% of the population, and are often asymptomatic and diagnosed incidentally. Approximately 20% of coronary anomalies have potential for life-threatening complication, including myocardial infarction, arrhythmia, or sudden death early in life or during adulthood. We describe here a patient with nonsustained polymorphic ventriculer tachycardia with 0.5 mg iv atropine after mixed type vasovagal syncope in the tilt table test and left coronary artery originates from the right coronary artery ostium. Methods: A 43-year-old female was admitted to the emergency department with chest pain and syncope. She had cigarette smoking and history of CAD in her family as cardiovasculer risk factors. Her laboratory findings, cardiac enzymes, ECG, Echocardiography was normal. Her syncope occured when she was standing and she felt no preceding or accompanying tachyarrhthmia. Tilt table testing was performed and she became both hypotensive and bradicardic and she fainted. 0.5 mg iv atropine was given, then nonsustained polymorphic VT occured and it was terminated spontaneously. Results: Coronary angiogram was performed and LAD was originating from the RCA’s ostium. Computerized tomography was performed; LAD was originating from the RCA’s ostium and continued intramiyocardial between aorta and pulmonary artery and then passed to the anterior interventriculer groove. Myocardial perfusion scintigraphy was normal. ICD implantation was suggested, but the patient rejected. Conclusions: Coronary artery anomalies are not uncommon, may be diagnosed incidentally or with sudden cardiac death. Standard testing with ECG under resting or exercise conditions is unlikely to provide clinical evidence of myocardial ischemia and would not be reliable as screening tests. If life threatening arrhytmias are diagnosed, either ischemia should be corrected or ICD should be implanted.

PP-079 CONGENITAL ABSENCE OF THE LEFT CIRCUMFLEX CORONARY ARTERY Arif Suner 1 , Sedat Koroglu 2 , Abdullah Sokmen 2 , Murat Koleoglu 2 , Hakan Kaya 2 , Alper Bugra Nacar 2 1 Department of Cardiology, Osmaniye State Hospital, Osmaniye, Turkey 2 Department of Cardiology, Sutcu Imam University, Kahramanmaras, Turkey An anomalous course of coronary arteries is observed in approximately 0.3% to 1.3% of patients undergoing diagnostic coronary angiography and in approximately 1% of routine autopsy examinations. Congenital absence of left circumflex coronary artery (LCx) is a very rare vascular anomaly in which the artery fails to develop in the left atrioventricular groove. There have been reported a few cases in the literature with a reported frequency of only 0.003% in all patients who underwent coronary angiography. Most patients with a congenital coronary artery anomaly are asymptomatic; rarely, they may present with chest pain and may have myocardial ischemia or other life-threatening conditions. Herein, a 58 years old female in whom the absence of LCx is associated with superdominant right coronary artery (RCA) was reported. In this case, there were critical lesions in both RCA and left anterior descending artery. The presence of high-grade stenosis in the RCA caused severe myocardial ischemia which was equivalent to two-vessel coronary artery disease involving both the right and left circumflex coronary arteries. Thus, the patient was considered as three vessel disease because of the critical left anterior descending artery lesion and she was referred for coronary artery by-pass graft operation. Congenital absence of LCx is thought to have a benign outcome unless atherosclerotic coronary artery disease superimposes. Critical lesions of

Cardiology Posters / International Journal of Cardiology 140, Supplement 1 (2010) S1–S93

coronary artery, which compensate the absence of LCx, become more complex. There are no definitive criteria about this condition in the literature.

PP-080 A CASE OF ABSENT RIGHT AND PERSISTENT LEFT SUPERIOR VENA CAVA

S65

Results: He has been discharged with medical treatment and clinically follow up was recommended. Conclusions: Congenital coronary–pulmonary artery fistula is rare. The incidence ranges from 0.3% to 0.8% in patients who undergo coronary angiography. After the diagnose of coronary fistulae with coronary angiography. MDCT is usually used to confirm.

Taliha Oner, Murat Muhtar Yilmazer, Baris Guven, Savas Demirpence, Timur Mese, Vedide Tavli Izmir Dr. Behcet Uz Children’s Hospital, Department of Pediatric Cardiology, Izmir, Turkey

PP-082 ANOMALOUS ORIGIN OF THE RIGHT CORONARY ARTERY FROM THE LEFT SINUS OF VALSALVA ASSOCIATED WITH BICUSPID AORTIC VALVE

Case: A 10-yr-old male patient was admitted for chest pain to our hospital. The patient was free of other cardiac symptoms. At admission his blood pressure (100/70 mmHg) and heart rate (90 beat/minute) were normal. Physical examination revealed no pathologic findings. Biochemical and hematological parameters were in the normal range. Chest X-ray was normal. The electrocardiogram showed normal sinus rhythm, and there was no evidence of arrhytmia Transthoracic echocardiography revealed a large coronary sinus with absence of the right superior vena cava (RSVC). Suprasternal window of the TTE displayed a persitant left superior vena cava (PLSVC) which draining to the coronary sinus. Transesophageal echocardiography imaging of the midesophageal, bicaval view with the transducer steered through 90–120° suggested absence of the RSVC. To obtain more detailed information, cardiac angiography had been performed and absence of the R-SVC and presence of the PLSVC which draining to coronary sinus were identified. Conclusions: Absence of the RSVC in visceroatrial situs solitus is a rare event, occurring in 0.07%–0.13% of congenital cardiovascular malformations. The presence of PLSVC occurs in approximately 0.3% to 0.5% of the general population. An absent RSVC is always associated with a PLSVC, with a reported incidence of 0.1% In most of patients with PLSVC, RSVC is present. In most of cases LSVC drains into coronary sinus and hence the coronary sinus is enlarged. Bartram et al. reviewed 121 patients with persistent left SVC and absent RSVC. About half of these cases had no other congenital malformations. Most common congenital defects associated with combined great venous malformation were various types of atrial septal defects, endocardial cushion defects and tetralogy of Fallot. The hemodynamics of patients with agenesia of RSVC and isolated PLSVC connected to the right atrium is the same as that of healthy individuals, as a result, patients are mostly asymptomatic. However it may cause difficulties and serious complications in central venous catheterization, intracardiac electrode placement or during cardiopulmonary bypass. Detailed and accurate echocardiographic studies may identify this rare combined congenital defect, hence preventing future complications during invasive procedures. The diagnosis of persistent left superior caval vein once required diagnosis by catheterisation or angiocardiography or both, but now non-invasive diagnosis by echocardiography is possible.

Mustafa Cetin 1 , Ozgul Ucar 1 , Hulya Cicekcioglu 1 , Lale Pasaoglu 2 , Zehra Cetin 1 1 Ankara Numune Education and Research Hospital, Department of Cardiology, Ankara, Turkey 2 Ankara Numune Education and Research Hospital, Department of Radiology, Ankara, Turkey

PP-081 BILATERAL CORONARY-PULMONARY ARTERY FISTULA WITH RECENT CORONARY ARTERY DISEASE Ahmet Karagoz, Ibrahim Kocaoglu, Cagin Ureyen, Menderes Tapsiz, Mehmet Ileri, Feridun Vasfi Ulusoy Department of Cardiology, Numune Research Hospital, Ankara, Turkey Objective: Coronary artery fistulae are abnormal communications between a coronary artery and a cardiac chamber or major vessel (vena cava, pulmonary veins, pulmonary artery). They are usually diagnosed by coronary arteriography. Clinical presentations are variable depending on the type of fistula, shunt volume, site of the shunt, and presence of other cardiac conditions. Most coronary-pulmonary artery fistulas are clinically and hemodynamically insignificant and are usually found incidentally. Methods: We present the cases of a 59-year-old man with bilateral coronary-pulmonary fistula involving two coronary arteries. He had history of recent acute anterior miyocardial infaction and PTCA + coronary stent were adminsterd to left anterior descending artery in 2007. He presented with typical chest pain and in coronary angiography his stent was intact. Tl-201 SPECT study showed fixed anterior perfussion defect probably due to recent anterior infarction. MDCT (multi detector computer tomography) also showed bilateral coronary-pulmonary artery fistula from both right and left coronary arteries via a dilated fistulous vascular malformation.

Objective: Coronary artery abnormalities are found in approximately 1% of coronary angiograms. The possibility of such an anomaly should be considered especially in young individuals with chest pain,syncope or exertional dyspnea. However an anomalous coronary artery can be encountered at any age group of patients with cardiac symptoms. Methods: A 79-years-old Caucasian female patient presented to the cardiology outpatient clinic with atypical chest pain unrelated to physical exercise which started one week ago. She had advanced age and hypertension as coronary risk factors. The twelve-lead electrocardiogram revealed sinus rhytm with T wave negativity through the leads V1-V3. On physical examination her blood pressure was 130/80 mmHg and pulse rate was 70 bpm. On cardiac auscultation a grade 3/6 systolic ejection murmur could be heard at the aortic area. Transthoracic echocardiogram detected a calcified bicuspid aortic valve with mild stenosis and regurgitation. No left ventricular wall motion abnormality was present. The patient could not perform a treadmill exercise test because of severe bilateral degenerative osteoarthritis of the knees. Therefore a dipyridamole nuclear scintigraphic stress testing was planned. However, headache, nausea and flushing occured after dipyridamole administration and the test was cancelled. We decided to continue diagnostic work-out with 16 slice multislice cardiac computed tomography (MSCT). Results: MSCT revealed calcified non obstructive coronary plaques and abnormal origin of the right coronary artery (RCA) from the left sinus of Valsalva. Due to the advanced age of the patient and recent onset of symptoms, we thought that this congenital coronary abnormality was unresponsible for the clinical condition. We started medical therapy with acetylsalicylic acid, betablocker and statin. The patient is free of symptoms for one year. Conclusions: Anomalous origin of the RCA from the left sinus of Valsalva is a rare coronary artery anomaly with an incidence of 0.003% to 0.9% in patients undergoing coronary angiography. RCA courses between the ascending aorta and the pulmonary trunk. This anomaly can cause sudden death in young individuals. A group of patients can survive to late adulthood without symptoms. Its association with a bicuspid aortic valve is extremely rare with only a few cases being reported previously. In fact, the probability of anomalous coronary artery patterns increases as the aortic valve or aortic root deviates from its normal structure.Because of the unusual origin and noncircular orifis of the anomalous vessel, selective catheterization and percutaneous coronary intervention can be technically challenging.

PP-083 GIANT PULMONARY ARTERY ANEURYSM DUE TO UNCORRECTED ATRIAL SEPTAL DEFECT EVALUATED BY MULTIDETECTOR COMPUTED TOMOGRAPHY Enbiya Aksakal 1 , Mecit Kantarci 2 , Huseyin Senocak 1 1 Department of Cardiology, Faculty of Medicine, Ataturk University, Erzurum, Turkey 2 Department of Radiology, Faculty of Medicine, Ataturk University, Erzurum, Turkey Objective: Pulmonary artery aneurysm (PAA) is a rare clinical entity that may be either congenital or acquired. Identification of enlargement of the