Validity and reliability of the Turkish version of the EmPHasis-10 questionnaire in patients with pulmonary hypertension.

RATIONALE, AIMS, AND OBJECTIVES: The aim of the study was to evaluate the reliability and validity of the Turkish version of the EmPHasis-10 questionnaire to ensure cultural adaptation. METHODS: This study involved translation, back translation, and cross-cultural adaptation. One hundred and one patients who were diagnosed as having pulmonary hypertension (PH) for at least 6 months were evaluated using the Turkish version of EmPHasis-10. Turkish version of the Minnesota Living with Heart Failure Questionnaire (MLHFQ) was used as gold standard to assess the validation of the Turkish version of the EmPHasis-10 questionnaire. Relationship between MLHFQ and EmPHasis-10 was analysed using Spearman correlation analysis to assess the validation. Cronbach alpha (internal consistency) and exploratory factor analyses were used to assess the questionnaire's reliability. RESULTS: The statistical analysis showed that the EmPHasis-10 questionnaire showed a high validity with MLHFQ (r = 0.85) (P = 0.001). Reliability analysis showed that EmPHasis-10 had a high level of Cronbach alpha (α = 0.98) and internal consistency (ICC = 0.97). CONCLUSIONS: The Turkish version of EmPHasis-10 is a quality of life questionnaire specific to PH. It has a high-level validity and reliability questionnaire that can be used by researchers and physicians.

Preliminary results from a nationwide adult cardiology perspective for pulmonary hypertension: RegiStry on clInical outcoMe and sUrvival in pulmonaRy hypertension Groups (SIMURG).

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.

Expert panel on cost analysis of atrial fibrillation.

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.

[Prostanoids in the treatment of pulmonary arterial hypertension]. / Pulmoner arteriyel hipertansiyon tedavisinde prostanoidler.

Prostacyclin, endothelin-1, and nitric oxide pathways are involved in the pathogenesis of pulmonary arterial hypertension. This devastating disease of the pulmonary vasculature is associated with vasoconstriction, thrombosis and proliferation, and this may be partly due to lack of endogenous prostacyclin secondary to prostacyclin synthase downregulation. Prostanoids (prostacycin analogues) are potent vasodilators and possess antiaggregant, antiinflammatory and antiproliferative properties. The first agent to be approved for the treatment of pulmonary arterial hypertension was epoprostenol. In the last decade other prostanoids (treprostinil, iloprost) has been approved for the treatment of pulmonary arterial hypertension.

[Phosphodiesterase type 5 inhibitors in the treatment of pulmonary arterial hypertension]. / Pulmoner arteriyel hipertansiyon tedavisinde fosfodiesteraz -5 inhibitörleri.

The pathology of pulmonary arterial hypertension (PAH) is characterized by vascular vasoconstriction, smooth muscle cell proliferation, and thrombosis. Experimental studies have shown the beneficial effect of phosphodiesterase type 5 (PDE-5) inhibitors on pulmonary vascular remodeling and vasodilatation. Randomized clinical trials in monotherapy or combination therapy have been conducted in PAH with sildenafil and tadalafil which significantly improve clinical status, exercise capacity and hemodynamics of PAH patients. Combination therapy of PDE-5 inhibitors with prostacyclin analogs and endothelin receptor antagonists may be helpful in management of PAH. The third PDE-5 inhibitor, vardenafil, is currently being investigated in PAH. Side effects are usually mild and transient and include headache, flushing, nasal congestion, digestive disorders, and myalgia.

[Pulmonary hypertension: diagnosis and clinical classification]. / Pulmoner hipertansiyon: tani ve klinik siniflama.

Pulmonary hypertension (PH) is a complex disease with limiting the physical activity, life expectancy significantly and requires multidisciplinary approach. In recent years, a dramatic increase was observed in the understanding and management of the disease. The first clinical classification of PH was made in Evian (France) in 1973 and the last clinical classification of PH was made in Dana Point (USA) in 2008. Diagnosis and clinical classification of PH is discussed in this review.

Stunning of the left atrium after conversion of atrial fibrillation: predictor for maintenance of sinus rhythm?

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.

Isolated ventricular noncompaction.

Isolated ventricular noncompaction of myocardium is a rare congenital disease due to an arrest of myocardial morphogenesis during foetal development. It is characterized by a thin compacted epicardial and an extremely thickened endocardial layer with prominent trabeculations and deep intertrabecular recesses. The persistence of myocardial noncompaction is usually an associated anomaly in patients with congenital left or right ventricular outflow tract obstruction. However, isolated noncompaction of myocardium is not associated with any factors that would explain it apart from the foetal arrest of compaction of the ventricular myocardium. The disease results in systolic and diastolic ventricular dysfunction, systemic embolism and ventricular arrhythmias. We describe a case of isolated noncompaction of the ventricular myocardium in a 20-year-old man who presented initially with ventricular tachycardia.

Right ventricular and tricuspid valve function in patients with two ventricular pacemaker leads.

UNLABELLED: Existing data on the effect of retained pacemaker leads on right ventricular (RV) and tricuspid valve function is limited. OBJECTIVE: In this echocardiographic study we investigated the long-term effect of retained ventricular leads on RV and tricuspid valve function in patients with permanent pacemakers. Forty patients, 18 with two (group I) and 22 with one (group II) ventricular lead were assessed echocardiographically at an average of 39 months after the second lead implantation in group I and 80 months after the lead implantation in group II. The sum of the lead body diameter in group I was significantly greater than the body diameter in group II (P < 0.000). There was no significant difference between the groups with respect to chamber diameters and ventricular or valvular functions. The distributions of the different tricuspid regurgitation (TR) grades were similar, with the majority of patients in both groups having mild TR. Retained second pacemaker leads do not have an additional negative effect on right ventricular and tricuspid valve function.

A patient with bicuspid aorta and intercoronary continuity: a rare and variant of coronary circulation.

Bidirectional 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.

A rare cause of mitral insufficiency: left atrial anomalous band.

Anomalous muscular bands have been seen in the left ventricle and right and left atria. Although the significance of left ventricular false tendons and right atrial anomalous muscular bands have been reported previously, the importance of anomalous muscular bands observed in the left atrium is not clearly defined. They have been found to be associated with Chiari's network, patent foramen ovale, and supraventricular arrhythmias. We describe a left atrial anomalous band causing mitral valve prolapse and mitral regurgitation.

The safety of low-molecular weight heparins for the prevention of thromboembolic events after cardioversion of atrial fibrillation.

Transesophageal echocardiography (TEE) guided early cardioversion (CV) in conjunction with short-term anticoagulation has been shown to be safe, and an alternative to prolonged conventional anticoagulation therapy. Recently, low molecular weight heparins (LMWHs) have been used successfully as an alternative to standard heparin therapy obviating the need for hospitalization and APTT monitoring. The aim of this study was to determine the feasibility and safety of TEE guided early cardioversion in conjunction with short-term LMWH use in patients with nonvalvular atrial fibrillation (NVAF). The study group consisted of 172 consecutive patients with NVAF. Before TEE, 90 patients received LMWH (Dalteparin 2 x 5,000U) and 82 patients received standard heparin (UFH) (5,000U bolus followed by infusion to raise APTT to 1.5 times control). TEE was performed and the left atrium and left atrial appendage were examined thoroughly for the presence of thrombus. One patient from each group was excluded due to detection of a left atrial thrombus by TEE. Immediately after TEE, CV was attempted and warfarin was initiated. All patients received warfarin for one month after CV. In the LMWH group, 89 of 90 patients (98.9%) were successfully cardioverted. CV was successful in 97.5% of the patients in the UFH group. None of the patients experienced thromboembolic events during the four weeks after CV. TEE guided early CV in conjunction with short-term LMWH treatment is as safe as UFH for the prevention of thromboembolic events after CV.

Utility of dobutamine stress echocardiography in Kawasaki disease: a case report and review of the literature.

Dobutamine stress echocardiography (DSE) has become widely accepted in the evaluation of adult patients with coronary heart disease. There are new challenges about the use of DSE in the pediatric age group to document ischemia. DSE can demonstrate ischemia noninvasively in Kawasaki disease (KD) patients who are candidates for coronary angiography. We wanted to assess the feasibility and the physiologic responses of DSE in a KD patient with coronary aneurysm. The patient had no ischemia in DSE, which was confirmed by coronary angiography showing no stenosis. The literature about DSE use in KD is reviewed.

Predictors for maintenance of sinus rhythm after cardioversion in patients with nonvalvular atrial fibrillation.

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.

The efficacy of valsartan in essential hypertension and its effects on left ventricular hypertrophy.

Recent studies demonstrate that, treatment with angiotensin-converting enzyme (ACE) inhibitors and angiotensin-II receptor blockers may decrease left ventricular hypertrophy (LVH) in hypertensive patients. We wanted to assess the efficacy of valsartan on echocardiographic variables of LVH in 30 mild to moderate hypertensive patients. Valsartan was found effective in controlling hypertension, also echocardiographic indices of LVH such as interventricular septum and posterior wall thickness were reduced and left ventricular mass decreased significantly. Thus valsartan favorably influences cardiac structure in hypertensive patients.

Quadricuspid aortic valve abnormality associated with aortic stenosis and aortic insufficiency.

Quadricuspid aortic valve is a rare congenital malformation of the aortic valve. Transthoracic 2-dimensional echocardiography can delineate aortic valve and diagnose most quadricuspid aortic valves. This article is a report of a quadricuspid aortic valve associated with aortic stenosis and aortic insufficiency diagnosed by transthoracic echocardiography.