Pulmonary arterial hypertension and systemic sclerosis relation: a single centre experience.

AIM: In systemic sclerosis (SSc), this single-centre study aimed to define the frequency and association of pulmonary arterial hypertension (PAH), occurring either alone in SSc-PAH or together with interstitial lung disease (ILD-PH). MATERIAL-METHODS: SSc cases between the years 1990-2011 were reviewed, retrospectively. Patients' clinical, laboratory findings, Modified Rodnan Skin Score and Medsger score, 6-minute walk distance (6MWD), carbon monoxide diffusion test (DLCO), echocardiography, thorax HRCT, and right heart catheterisation findings were recorded. RESULTS: One hundred and forty-one cases (F/M:124/17, diffuse cutaneous SSc (DcSSc)/limited cutaneous SSc (LcSSc): 84/57) were included in the study with the mean age of 52.70±15.17 years and disease duration of 107.07±99.44 months. PaO2, FEV1 and FVC were lower in DcSSc (p<0.05) as compared to LcSSc, but DLCO and 6MWD did not differ significantly, between the two forms. Ground glass opacity (64.7%) and interlobular septal thickening (58.8%) were the most frequent findings on HRCT of such subjects. PAH was detected in 34 subjects (24.1%). Seven of them had SSc associated PAH (SSc-PAH) and 27 ILD-PH. Both frequencies were similar between DcSSc and LcSSc. Mean sPAP was higher in SSc-PAH. CONCLUSION: PAH was observed in approximately one fourth of patients; therefore advanced cardio-pulmonary investigation should be routinely performed in the SSc patients' management.

Clinical efficacy, safety, tolerability, and survival outcome of long-term inhaled iloprost treatment in the management of pulmonary arterial hypertension: Data from prospective multicenter observational OPTION study.

OBJECTIVE: To evaluate clinical efficacy, safety and tolerability of long-term inhaled iloprost treatment in the daily practice for the management of pulmonary arterial hypertension (PAH). METHODS: A total of 115 patients with PAH on inhaled iloprost treatment were included. New York Heart Association (NYHA) functional class, brain natriuretic peptide (BNP) and N-terminal pro-B-type natriuretic peptide (NT-proBNP) levels, and 6-minute walk distance (6MWD) were recorded at baseline and at 3rd to 24th month visits. Safety and tolerability of iloprost treatment were also evaluated during follow-up, as were the survival, clinical worsening, and the related risk factors. RESULTS: The treatment was associated with an increase in the percentage NYHA functional class II (from 0.0% at enrolment to 36.2% at 24th month visit) patients but no significant difference was noted in 6MWD values. Clinical worsening was observed in 63.5% patients, while survival rate was 69.6%. NT-proBNP levels were significantly higher in non-survivors than in survivors (p=0.042). Cox regression analysis revealed the association of female sex [odds ratio (OR)=0.318; 95% confidence interval (CI), 0.128-0.792; p=0.014] and scleroderma-related PAH (OR=0.347; 95% CI, 0.140-0.860; p=0.022) with significantly lower risk (3.14 fold and 2.88 fold, respectively) of mortality. CONCLUSION: Our findings indicate favorable efficacy, safety, and tolerability of long-term iloprost treatment in the management of PAH, whereas improved NYHA functional class was not accompanied with a significant change in 6MWD values. Patient age was a risk factor for clinical worsening, while female sex, scleroderma subtype, and lower NT-proBNP levels were associated with significantly lower mortality risk.

Heart transplant recipient survivor from COVID-19: The first case of Turkey.

Any highly infectious and rapidly spreading disease is a primary concern for immunocompromised solid organ transplant recipients. The number of data about the spectrum of clinical illness, the treatment modalities, and the outcomes of COVID-19 in this vulnerable population is scant and still remains empirical. Herein, we report the first COVID-19 case of a heart transplant recipient in Turkey who presented with fever, postnasal discharge, and myalgias for two days. The possibility of lung involvement was ruled out by thoracic computed tomography. Despite stable vital signs, we reduced the intensity of immunosuppressive therapy and maintained home self-isolation promptly. We also commenced a five-day course of hydroxychloroquine 200 mg q12h initially. After confirmation of real-time reverse-transcriptase-polymerasechain- reaction testing of the nasopharyngeal swab positive for COVID-19, the patient was hospitalized. After a loading dose of favipiravir 1,600 mg b.i.d., the patient received a five-day course of favipiravir 600 mg q12h. He was discharged with cure after 23 days of hospital isolation and treatment. In conclusion, treatment process can be affected by the daily electrocardiography, hand-held portable echocardiography, myocardial injury markers, and pulse oximeter for selfmonitoring in the follow-up of previous heart transplant recipients suffering from COVID-19. The lack of treatment protocols in the solid organ transplant recipients with COVID-19 infection and the controversies about the protective effect of immunosuppression invite a global and update discussion.

Changing patterns of patient characteristics in the cath lab from 1998 to 2006: a single-centre retrospective analysis.

BACKGROUND: There are few data on the change in the profile of cath lab patients over long time intervals. METHODS: We retrospectively analysed our cath lab records, patient charts, in terms of demographic variables, clinical and laboratory characteristics in a thousand patients (499 patients in 1998 January-March, 501 patients in 2006 January-March). RESULTS: Mean age was significantly higher in the 2006 cohort (57.5 +/- 11 vs. 62.2 +/- 10.8, P < 0.001). Gender was similar in both cohorts (men 68.5% vs. women 69.9%, P = 0.65). Both hypertension and diabetes mellitus were more prevalent in the 2006 cohort (613% vs. 49.3 and 30.3 vs. 17.6 respectively, P < 0.001 for both). Smoking rates (past or active) did not differ between the cohorts. Rates of normal or near normal coronary angiograms were somewhat elevated in both cohorts (36.7% in 1998 and 39.1% in 2006). Rates of multivessel disease (2-3 vessel disease) tended to increase and rates of single-vessel disease tended to decrease from the 1998 cohort to the 2006 cohort (27.7% to 34.4% and 35.7% to 26.7%, P = 0.006). The number of percutaneous coronary interventions (PCI) performed increased with a borderline statistical significance in the 2006 cohort (32% vs. 38.5%, P = 0.053). CONCLUSIONS: Our data indicates that today interventional cardiologists face an older and more severely diseased cath lab patient population compared with a decade earlier. In comparison with 1998, more PCIs were performed.

Heart failure due to aorto-right atrial fistula and successful treatment with percutaneous closure.

Aorto-atrial fistula is a rare anomaly of the heart that may be the result of congenital or acquired conditions, such as bacterial endocarditis, paravalvular abscess, aortic dissection, or a complication of cardiac surgery. A 50-year-old female patient presented at the clinic with the complaint of dyspnea and abdominal distention due to ascites. On admission, her functional capacity was New York Heart Association (NYHA) class III. A physical examination revealed a systolic murmur, which was best audible in the right parasternal side, ascites, and hepatomegaly. An aorto-right atrial fistula was detected using transthoracic echocardiography and confirmed with transesophageal echocardiography and aortography. The patient had a history of previous cardiac surgery, anticoagulant use, and heart failure; therefore, percutaneous intervention was preferred to surgery as a result of the high surgical risk. A successful closure of the fistula was performed with an Amplatzer Duct Occluder II device. The patient demonstrated a dramatic response to the treatment, resulting in a decrease in the ascites and halving of her diuretic dose within 1 week. Her functional capacity improved to NYHA class II, and right atrial pressure decreased to 8 mmHg after a month.

Natriuretic Peptides in Clinical Practice.

Natriuretic peptides have long been introduced into clinical practice. These biomarkers have certainly been shown to provide useful information in the diagnosis, prognosis and risk stratification in heart failure and also may have a role in the guidance of heart failure therapy. Although, there are some limitations in using of these markers such as lack of specificity, aging, renal dysfunction or obesity, among the huge number of candidates for heart failure biomarkers, only natriuretic peptides are currently widely used in daily clinical practice in heart failure. Recent heart failure guidelines recognize natriuretic peptides as an essential tool in the new diagnostic and therapeutic algorithms. Furthermore, natriuretic peptides are not only used in the diagnosis or prognosis of heart failure, but also these biomarkers are referred to have some potential role in primary prevention, cardio-oncology, advanced heart failure, assessment of response to cardiac resynchronization therapy, pulmonary arterial hypertension, acute coronary syndromes, atrial fibrillation and valvular heart disease. In this article, natriuretic peptides have been reviewed for their updated information and new recommendations in heart failure and also potential role of these biomarkers in the management of various clinical conditions have been addressed in the form of expert opinion based on the available data in the literature.

Angiographic restenosis in ephesos coronary stents: experience from a large medical center in Ankara, Turkey.

Coronary stent restenosis, which emerges in late periods after implantation, has not been completely abolished. Our aim was to investigate the restenosis rates of Ephesos coronary stents. In all, 96 patients (66 men) with 135 Ephesos coronary stents were included. Control angiograms were performed after 160 +/- 60 days. Quantitative coronary analysis was performed during the procedure and control angiogram. The stents were divided into 2 groups according to the presence or absence of restenosis. Groups were compared with clinical and angiographic variables. Restenosis was observed in 31 (23%) of 135 stents. Preprocedure percent diameter stenosis was higher (P = .02), whereas minimum lumen diameter ( P = .02), mean age (P < .001), and hypertension incidence ( P = .043) was less, and there was a trend toward smaller stent size ( P = .054) in the restenosis group. By multivariate analysis, age <50 years (P < .001) and stent size <3.0 mm (P = .016) were independent predictors of restenosis. Ephesos coronary stents seems to have acceptable restenosis rates.

Intravascular volume dependency of left ventricular mass calculation by two-dimensional guided M-mode echocardiography.

BACKGROUND: Increased left ventricular mass (LVM) is an independent risk factor for cardiovascular morbidity and mortality, and may be used for risk stratification. Two-dimensional echocardiography, the most commonly used technique for estimation of LVM, uses the third power of the left ventricular internal diameter (LVID) for the calculation. OBJECTIVES: To determine whether a decrease in intravascular volume after dialysis may cause inaccurate estimation of LVM by echocardiography. METHODS: Thirty-eight patients undergoing hemodialysis due to chronic renal failure constituted the study group (14 women [37%] and 24 men [63%], mean age +/- SD 38.7+/-10.9 years). LVID, and interventricular and posterior wall thicknesses were measured by two-dimensionally guided M-mode echocardiography. Stroke volume and cardiac output were calculated using left ventricular outflow tract diameter and the pulsed-wave Doppler time-velocity integral obtained from left ventricular outflow tract. LVM was calculated by using Devereux's formula, and was indexed for body surface area and height. All echocardiographic parameters were measured or calculated before and after dialysis (on the same day), and then compared. RESULTS: There were no significant changes in wall thickness; however, LVID, LVM, the LVM/body surface index and the LVM/height index significantly decreased after dialysis (P<0.001 for each parameter). There was a significant correlation between the change in LVID and the change in LVM (P<0.001, r=0.59). Stroke volume and cardiac output also decreased significantly after hemodialysis (P<0.001 for each parameter). CONCLUSIONS: Intravascular volume-dependent change in LVID causes inaccurate estimation of LVM, so volume status should be kept in mind, especially in serial assessment of LVM.

Gadolinium:nonionic contrast media (1:1) coronary angiography in patients with impaired renal function.

This study was to test safety and efficacy of 1:1 mixture of gadolinium:nonionic contrast media in avoiding contrast nephropathy during coronary angiography in patients with renal dysfunction. Although "off label" for x-ray angiography, gadolinium has drawn attention for its potential to avoid contrast nephropathy during coronary angiography. Initial data seem promising. Patients with a baseline creatinine of 1.5 mg/dl or more were included. In order to minimize contrast nephropathy risk, all patients were thoroughly hydrated and treated by N-acetylcysteine. After coronary angiography and/or percutaneous coronary intervention, renal function tests were remeasured on days 1, 2, and 3. A rise of 0.5 mg/dl or more in creatinine value in the following 3 days or the need for dialysis were considered as a contrast nephropathy event. Twenty-six patients were enrolled in this study. Ten were women and 16 were men. The mean age was 65.7 +/- 11. Baseline creatinine value was 2.47 +/- 0.74 mg/dl. The total amount of contrast medium used on 1:1 fashion was 57.1 +/- 27.2 ml. No procedure-related cardiac complication or contrast nephropathy event occurred in this study. Although there was a slight loss in image quality, we felt that the results were adequate for interpretation. Although "off label," using gadolinium contrast media in a 1:1 mixture with standard nonionic low osmolar contrast media seems to be a viable option in decreasing the likelihood of contrast nephropathy. Further evaluation appears to be warranted.

Sustained long-term benefit of sildenafil in primary pulmonary hypertension.

Primary pulmonary hypertension is a rare disease with an ominous prognosis for which new therapeutic options are being developed. Recently, there have been case reports indicating that sildenafil may be of benefit in short- to midterm follow-up. However, long-term clinical data of sildenafil in primary pulmonary hypertensive patients is lacking. We report a patient with primary pulmonary hypertension treated with sildenafil 200 mg/day for 50 months with a sustained clinical response.

Altered coronary flow properties in diffuse coronary artery ectasia.

OBJECTIVES: The purpose of this study was to investigate coronary blood flow properties in patients with diffuse coronary artery ectasia (CAE) associated with exercise-induced myocardial ischemia. METHODS: Seventeen patients with diffuse CAE and without coexisting coronary artery stenosis were enrolled in the study (CAE group). CAE was defined as luminal dilatation 1.5 to 2 times that of the adjacent normal coronary artery segment or the diameter of the corresponding coronary artery of the control group when there was no normal segment. The age- and sex-matched control group (n = 20) comprised patients with normal epicardial coronary arteries. Coronary blood flow velocities were obtained invasively by use of Doppler scanning flow wire. Coronary flow reserve (CFR) was measured by administration of intracoronary papaverine as the hyperemic stimulus. Volumetric coronary blood flow was estimated by multiplying the velocity time integral of coronary blood flow with the cross-sectional area of the coronary artery and the heart rate. RESULTS: Fifteen patients with CAE, but none of the patients in the control group, had electrocardiographic signs of myocardial ischemia at peak exercise on ergometry. Baseline average peak velocities (APVs) of coronary blood flow were similar in the 2 groups. Peak hyperemic APVs of coronary blood flow were lower in the CAE group than in the control group (17.5 +/- 7.4 cm/s vs 41.5 +/- 12.6 cm/s, respectively, P <.001). Volumetric coronary blood flow was significantly higher in the CAE group than in the control group, both at rest and at hyperemia (146.3 +/- 71.2 cm3/min vs 45.1 +/- 16.1 cm3/min, respectively, P <.001, and 202 +/- 87.3 cm3/min vs 104.1 +/- 37.6 cm3/min, respectively, P <.003). The mean CFR of the CAE group was significantly reduced compared with that of the control group (1.51 +/- 0.31 vs 2.67 +/- 0.52, respectively, P <.001). CONCLUSIONS: The CFR is significantly reduced in patients with diffuse CAE compared to a matched control group. Although volumetric coronary blood flow is significantly higher in CAE, microcirculatory dysfunction that is reflected as depressed CFR may be the underlying cause of exercise-induced myocardial ischemia.

Sildenafil in primary pulmonary hypertension--is there a subset of patients who respond favourably?

Recently, case reports of patients with primary pulmonary hypertension (PPH) treated with sildenafil demonstrated encouraging results. The mechanism proposed is a relatively selective pulmonary vasodilation via increased levels of cGMP because of the inhibition of phosphodiesterase type 5. Two siblings with a similar medical history, severe symptoms and elevated levels of pulmonary artery pressures were diagnosed with PPH after a thorough diagnostic work-up. Both patients were treated with coumadin, sildenafil, furosemide, spironolactone and digoxin. One of the patients had no improvement during the hospital course and died two months after discharge. The other patient improved dramatically during the hospital course, and this improvement was sustained. At the three-month follow-up control, she was much improved in terms of clinical status and echocardiographic findings.

Effect of transvenous cardiac resynchronization therapy device implantation on cardiac troponin I release.

BACKGROUND: Pacemaker and implantable cardioverter defibrillator (ICD) implantation increases cardiac troponin I (cTnI) levels which indicates myocardial injury. During implantation of a cardiac resynchronization therapy (CRT) device, balloon inflation for coronary sinus (CS) venogram, cannulation of CS side branch, and electrode advancement may interfere with CS drainage and, hence, may decrease the washout of toxic metabolites from the heart. Thus, CRT implantation may further increase cTnI levels. In this study, we investigated the effects of CRT implantation on cTnI release. METHODS: We included 10 patients (mean age = 57 +/- 15 years) in whom a successful transvenous CRT system was implanted (CRT group). Twenty patients (mean age = 65 +/- 10 years) who underwent a transvenous pacemaker or ICD implantation were included as the control group. Blood samples for cTnI were drawn at baseline and at six, 12, 18, and 24 hours thereafter. RESULTS: Baseline median cTnI levels were similar in CRT and control groups (0.03 ng/mL vs 0.02 ng/mL, respectively; P = 0.1). Postoperative cTnI levels during 24 hours were significantly higher in the CRT group (P < 0.05) by two-way repeated measures of analysis of variance. Post hoc analysis revealed that cTnI levels were higher at the 6th, 12th, 18th, and 24th hours compared to baseline levels (P < 0.001, P < 0.001, P < 0.01, and P < 0.01, respectively). There was a significant difference in the area under the curves (AUCs) of cTnI measurements (1.79 hr.ng/mL in the CRT group and 0.78 hr.ng/mL in the control group, P < 0.05). CONCLUSION: Postoperative cTnI levels were higher after CRT implantation than simple pacemaker/ICD implantation. This may be due to CS manipulation during CRT implantation.

Two cases of quadricuspid aortic valve.

Quadricuspid aortic valve is a rare congenital malformation of the aortic valve. Most cases are discovered incidentally at autopsy or at aortic valve replacement. Recent advances in echocardiography epecially transesophageal echocardiography led to diagnosis of more cases before surgery. Two cases of quadricuspid aortic valve are presented.