Exercise intolerance is the main characteristic of pulmonary arterial hypertension (PAH). The six-minute walk test (6MWT) and cardiopulmonary exercise test are widely used in assessing exercise capacity of PAH patients. Six-minute walk distance (6MWD) has been specified as the main clinical outcome in PAH and has been used as the primary end-point in many studies conducted for new PAH treatments. Using 6MWD as the end-point in clinical studies has many advantages. 6MWT is an inexpensive, easily applicable, and repeatable standardized test that is well-tolerated by PAH patients. Moreover, it is a valid measure of symptomatic improvement. It is correlated with variables of maximal cardiopulmonary exercise test as a measure of submaximal exercise capacity and disease severity markers such as functional class and pulmonary hemodynamics. It is widely used in clinical practice together with other invasive and non-invasive disease markers in assessing disease progression and response to treatment. In addition, it has prognostic importance and is a good prognostic marker. On the other hand, there are limitations to the use of 6MWD as the primary end-point in PAH treatment. It has decreased sensitivity in individuals with less severe disease and high 6MWD at baseline and decreased adequacy in assessing the effects of treatment in patients who are still under PAH treatment. Despite the limitations, 6MWD plays a key role in the evaluation and management of PAH patients.

Recurrence of atrial fibrillation (AF) after cardioversion (CV) to sinus rhythm (SR) is determined by various clinical and echocardiographic parameters. Transesophageal echocardiographic (TEE) parameters have been the focus of clinicians' interests for restoring and maintaining SR. This study determined the clinical, transthoracic, and TEE parameters that predict maintenance of SR in patients with nonvalvular AF after CV. We enrolled 173 patients with nonvalvular AF in the study. TEE could not be performed in 26 patients prior to CV. Twenty-five patients had spontaneously CV prior to TEE. Six patients were excluded because of left atrial (LA) thrombus assessed by TEE. CV was unsuccessful in 6 patients. The remaining 110 consecutive patients (56 men, 54 women, mean age 69 +/- 9 years), who had been successfully cardioverted to SR, were prospectively included in the study. Fifty-seven (52%) patients were still in SR 6 months after CV. Age, gender, the configuration of the fibrillation wave on the electrocardiogram, pulmonary venous diastolic flow, and the presence of diabetes, hypertension, coronary artery disease, mitral annulus calcification, and mitral valve prolapse (MVP) did not predict recurrence. Duration of AF, presence of chronic obstructive pulmonary disease (COPD), LA diameter, left ventricular ejection fraction (EF), left atrial appendage peak flow (LAAPF), LAA ejection fraction (LAAEF), pulmonary venous systolic flow (PVSF), and the presence of LA spontaneous echo contrast (LASEC) predicted recurrence of AF 6 months after CV. In multivariate analysis, LAAEF < 30% was found to be the only independent variable (P < 0.0012) predicting recurrence at 6 months after CV in patients with nonvalvular AF. LAAEF more than 30% had a sensitivity of 75% and a specificity of 88% in predicting maintenance of SR 6 months after CV in patients with nonvalvular AF. In conclusion, TEE variables often used to determine thromboembolic risk also might be used to predict the outcome of CV.

Objective:The present study was designed to evaluate the characteristics of pulmonary hypertension (PH) and adult cardiology practice patterns for PH in our country.Methods:We evaluated preliminary survey data of 1501 patients with PH (females, 69%; age, 44.8±5.45) from 20 adult cardiology centers (AdCCs).Results:The average experience of AdCCs in diagnosing and treating patients with PH was 8.5±3.7 years. Pulmonary arterial hypertension (PAH) was the most frequent group (69%) followed by group 4 PH (19%), group 3 PH (8%), and combined pre- and post-capillary PH (4%). PAH associated with congenital heart disease (APAH-CHD) was the most frequent subgroup (47%) of PAH. Most of the patients’ functional class (FC) at the time of diagnosis was III. The right heart catheterization (RHC) rate was 11.9±11.6 per month. Most frequently used vasoreactivity agent was intravenous adenosine (60%). All patients under targeted treatments were periodically for FC, six-minute walking test, and echo measures at 3-month intervals. AdCCs repeated RHC in case of clinical worsening (CW). The annual rate of hospitalization was 14.9±19.5. In-hospital use of intravenous iloprost reported from 16 AdCCs in CWs. Bosentan and ambrisentan, as monotreatment or combination treatment (CT), were noted in 845 and 28 patients, respectively, and inhaled iloprost, subcutaneous treprostinil, and intravenous epoprostenol were noted in 283, 30, and four patients, respectively. Bosentan was the first agent used for CT in all AdCCs and iloprost was the second. Routine use of antiaggregant, anticoagulant, and pneumococcal and influenza prophylaxis were restricted in only two AdCCs.Conclusion:Our nationwide data illustrate the current status of PH regarding clinical characteristics and practice patterns.

Objective: To estimate total cost of atrial fibrillation (AF) management concerning acute coronary syndrome, heart failure, stroke and drug related adverse events with respect to clinical practice and available guidelines.Methods: This cost analysis study was based on identification of total costs related to management of acute coronary syndrome, heart failure, stroke and the drug related adverse events in patients with AF based on standardized questionnaire forms filled by experts according to their daily clinical practice and also to ACCF/AHA/ESC guidelines. Total cost included cost items related to treatment, healthcare resources utilization, and diagnostic test and consultations. Results: The yearly cost of acute coronary syndrome per patient was 5.478.43 TL according to expert's view reflecting real clinical practice whereas it was 11.319.44 TL when calculation was based on recommendations in the guidelines. The average total cost of heart failure was 4.523.74 TL according to expert's view whereas it was 2.925.86 TL based on guidelines. The average total cost of stroke was 5.719.25 TL according to expert's view but 7.931.18 TL based on guidelines. Among drug related adverse events, only those related to cardiac adverse events were estimated to be higher according to expert view as compared to guideline recommendations (288.65 vs. 150.99 TL). Conclusions: Reflecting the treatment algorithms in the management of AF and related adverse events, our findings seem to emphasize the extra burden on health economics posed by patients suffering from the uncontrolled disease. (Anadolu Kardiyol Derg 2013; 13: 26-38) Key words: Atrial fibrillation, acute coronary syndrome, heart failure, stroke, adverse events, cost analysis Original Investigation Özgün Araşt›rma 26ÖZET Amaç: Atriyal fibrilasyon (AF) yönetimi toplam maliyetinin klinik uygulama ve kılavuzlar bazında hesaplanarak akut koroner sendrom, kalp yetersizliği, inme ve ilaça bağlı advers olaylar açısından maliyet verilerinin sağlanması. Yöntem: Bu maliyet analiz çalışması, AF hastalarında akut koroner sendrom, kalp yetersizliği, inme ve ilaca bağlı advers olaylar bazında toplam maliyetin, kendi klinik pratikleri ve ACCF/AHA/ESC kılavuzlarında yer alan öneriler doğrultusunda araştırıcılar tarafından doldurulan standart anket formları aracılığı ile hesaplanması yolu ile yürütüldü. Toplam tıbbi maliyet hesabına dahil edilen maliyet kalemleri tedavi, sağlık kaynakları kullanımı, tanısal testler ve konsültasyon kalemleri olarak belirlendi. Bulgular: Akut koroner sendrom için hesaplanan ortalama toplam maliyet, uzmanların günlük klinik pratiği yansıtan görüşlerine göre 5.478.43 TL iken kılavuzlar doğrultusunda 11.319.44 TL olarak hesaplandı. Kalp yetersizliği toplam maliyeti uzman görüşlerine göre 4.523.74 TL iken kılavuzlar doğrultusunda 2.925.86 TL olarak hesaplandı. İnme toplam maliyeti uzman görüşlerine göre 5.719.25 TL, kılavuzlara göre ise 7.931.18 TL olarak hesaplandı. İlaca bağlı advers olaylar içinde, yalnızca kardiyak advers olaylar için, uzman görüşl...

We describe the case of a 60yearold woman who presented with pulmonary artery sarcoma, a very rare tumor of the cardiovascular system. Her tumor was initially misdiag nosed as chronic pulmonary thromboembolism, and she underwent pulmonary endarter ectomy.Early P ulmonary artery (PA) sarcoma, a very rare tumor of the cardiovascular system, is often misdiagnosed as acute or chronic pulmonary thromboembolism because its clinical presentation and radiologic findings resemble those of thromboembolism.1,2 The prognosis is usually poor: the tumor is invasive and often involves vital structures such as the heart, which makes radical resection challenging. 3 We describe the case of a 60-year-old woman with PA sarcoma that was initially misdiagnosed. Case ReportIn February 2013, a 60-year-old woman presented at our emergency department with progressive dyspnea and palpitation. The dyspnea had started after a long bus ride. Her New York Heart Association (NYHA) functional class was II/III. On physical examination, she was positive for jugular venous distention. Auscultation revealed a grade 3/6 systolic ejection murmur in the left parasternal and 2nd intercostal areas. Her hemodynamic state was stable. Her electrocardiogram showed sinus rhythm and a pattern of right ventricular (RV) strain. The patient's cardiothoracic ratio was greater than 50%, and her natriuretic pro-brain peptide level was high. Transthoracic echocardiography (TTE) and computed tomography (CT) of the chest showed RV dilation with impaired function and a mass (2.7 × 1.3-cm) extending from the pulmonary valve into the right and left PAs and into all sub-branches, which suggested a pulmonary embolus (Figs. 1 and 2). Systolic PA pressure (sPAP) derived by Doppler echocardiography was 122 mmHg. Laboratory investigations for hypercoagulable disorders were within normal limits. Malignancy was ruled out by positron emission tomography-CT (PET-CT). Doppler studies of the lower-extremity veins were normal. A diagnosis of subacute pulmonary thromboembolism was made on the basis of the patient's clinical presentation and imaging results, and we began anticoagulation with heparin. Thrombolytic therapy was not started because of her stable hemodynamic condition.Subsequently, the patient was placed on warfarin therapy for 3 months and monitored. Her echocardiogram revealed a dilated RV with reduced systolic function and moderate tricuspid regurgitation, no decrease in the size of the mass, and normal left ventricular function. Her sPAP was 122 mmHg. Computed tomograms of her chest showed web-like filling defects in the pulmonary vasculature on the right and left lower lobes, arising from a pulmonary-valve condition that was consistent with

SUMMARYBidirectional flow in patients with normal coronary arteries is an indicator of intercoronary continuity, a rare variant of coronary circulation, distinct from collaterals. The case of an 18 year old Turkish male with bicuspid aorta and intercoronary artery is reported and different aspects of this interesting entity are emphasized. (Jpn Heart J 2004; 45: 153-155) Key words: Coronary angiography, Coronary circulation, Coronary anomalies, Bidirectional flow BIDIRECTIONAL coronary flow is frequently seen in patients with severe coronary stenosis and after coronary artery by-pass surgery. In patients with normal coronary arteries, it is an indicator of intercoronary continuity or the so called "coronary cascade". CASEAn 18 year old boy presented with exertional chest pain in the left arm. His past medical history was unremarkable. He had none of the classical risk factors for coronary artery disease. Physical examination revealed a 4/6 systolic ejection sound radiating to the carotids and a 2/6 diastolic murmur. ECG revealed sinus rhythm with signs of marked left ventricular hypertrophy. Echocardiography showed left ventricular dilatation and hypertrophy, a severely calcified aortic valve with severe regurgitation and a mean gradient of 52 and peak gradient of 75 mmHg. Transesophageal examination demonstrated a bicuspid aorta as the underlying pathology. Coronary angiography showed normal coronaries but during left coronary injection the distal part of the right coronary artery (RCA) was From the

SUMMARYAtrial fibrillation (AF) is a very common cardiac arrhythmia with an increased mortality in patients with heart failure. Whether the best therapeutic approach to these patients is to restore sinus rhythm or to adequately control the ventricular rate is still controversial. The aim of this study was to compare both strategies in patients with AF and nonischemic heart failure.One hundred and fifty-four patients with AF duration greater than 48 hours and nonischemic left ventricular dysfunction were randomized either to a rhythm (n = 84) or rate (n = 74) control group. The composite end points of the study were embolism, death, and exercise capacity.The average age of the patients was 61 ± 10 years in the rhythm control group and 58 ± 12 years in the rate control group (P = NS). The average follow-up period was 35 ± 21 months in the rhythm control group and 37 ± 19 months in the rate control group (P = NS). In the first year of the study, exercise capacity and left ventricular ejection fraction (LVEF) were improved in the rhythm control group compared to the exercise capacity and LVEF of the rate control group (P < 0.0001 and P = 0.0005, respectively).There were no statistically significant differences in the embolic event rate between the two groups (P = NS). The mortality rate, especially for death due to pump failure, was significantly higher in the rate control group at the end of the study (P < 0.0001).Restoring and maintaining sinus rhythm had a beneficial effect on mortality and exercise capacity in patients with nonischemic heart failure and AF. (Jpn Heart J 2004; 45: 591-601)

Recurrence of atrial fibrillation (AF) after cardioversion (CV) to sinus rhythm (SR) is determined by various clinical and echocardiographic parameters. The aim of this study was to determine the value of mitral inflow A-wave velocity, performed at 24 hours after CV in estimation of AF recurrence. The study group consisted of 187 consecutive patients with nonvalvular atrial fibrillation, who had been cardioverted to SR from 1998 to 2000. Transthoracic echocardiography was performed in all cases recruited for the study 24 hours after CV. Left atrial (LA) diameter, left ventricular ejection fraction, and mitral inflow A-wave velocity were measured. The patients were evaluated in five groups according to their recurrence time (<30 days, 31-90 days, 91-180 days, 181-365 days, and >365 days). Maintenance of SR was determined to have a negative linear correlation with age (r =-0.97, P = 0.006), LA diameter (r =-0.93, P = 0.02), and AF duration (r =-0.93, P = 0.02), while having a positive linear correlation with mitral inflow A-wave velocity (r = 0.96, P = 0.008). In the maintenance of sinus rhythm, age, LA diameter, and AF duration were not affected from the method of CV, while mitral inflow A-wave velocity was found to be affected with the method of CV. No relationship was determined between mitral inflow A-wave velocity and the maintenance of sinus rhythm in those performed electrical cardioversion, while frequency of recurrence was found to be higher in those with slow mitral inflow A-wave velocity who were performed pharmacological cardioversion (r = 0.89, P = 0.004). In conclusion, age, duration of AF, LA diameter, and the mitral inflow A-wave velocity can be used to predict the maintenance of SR after CV.