Results of surgical treatment of pulmonary artery sarcomas: Does histology affect survival?

Background: In this study, we aimed to describe our experience with primary pulmonary artery sarcoma in patients who underwent pulmonary endarterectomy and to evaluate clinical features, treatment, outcomes, and survival rates according to the histological subtypes of this malignant disease. Methods: Between March 2011 and May 2022, a total of 13 patients (7 males, 6 females; mean age: 52.6±13.0 years; range, 30 to 69 years) who underwent pulmonary endarterectomy and diagnosed with a pulmonary artery sarcoma were retrospectively analyzed. The diagnosis was confirmed histopathologically in all patients. Data including demographics, clinical characteristics, intra- and postoperative complications, length of hospital stay, morbidity, mortality, and short-term and long-term outcomes were recorded. Operative mortality was defined as death in the hospital or within 30 days of surgery. Results: Mortality was observed in one patient due to massive hemoptysis. Morbidity developed in two patients due to acute respiratory distress. Pulmonary vascular resistance improved significantly from 508 dyn/s/cm-5 to 191 dyn/s/cm-5 (p<0.004). All patients received chemotherapy following surgery. Median followup was 14 months. Median survival for the entire series was 18 months. One-year and three-year survival rates were 60.6% and 30.3%, respectively. Median survival for leiomyosarcomas (n=6) was seven months, while it was 44 months for intimal sarcomas (p=0.004). Three-year survival was 66.7% for intimal sarcomas and 0% for leiomyosarcomas. Conclusion: Pulmonary artery sarcoma may mimic chronic thromboembolic pulmonary hypertension. Patients with a suspected diagnosis of pulmonary artery sarcoma should be referred to expert pulmonary endarterectomy centers for surgery where a multidisciplinary team is available. Pulmonary endarterectomy has both diagnostic and therapeutic value and may improve survival and quality of life. Patients with intimal sarcoma have longer survival compared to those with leiomyosarcoma.

Risk Assessment Tool Implementation in Congenital Heart Disease-Associated Pulmonary Arterial Hypertension.

BACKGROUND: Risk assessment is recommended for patients with congenital heart disease-associated pulmonary arterial hypertension. This study aims to compare an abbreviated version of the risk assessment strategy, noninvasive French model, and an abridged version of the Registry to Evaluate Early and Long-term Pulmonary Arterial Hypertension Disease Management 2.0 risk score calculator, Registry to Evaluate Early and Long-term Pulmonary Arterial Hypertension Disease Management Lite 2. METHODS: We enrolled a mixed prevalent and incident cohort of patients with congenital heart disease-associated pulmonary arterial hypertension (n = 126). Noninvasive French model comprising World Health Organization functional class, 6-minute walk distance, and N-terminal pro-hormone of brain natriuretic peptide or brain natriuretic peptide was used. Registry to Evaluate Early and Long-term Pulmonary Arterial Hypertension Disease Management Lite 2 includes functional class, systolic blood pressure, heart rate, 6-minute walk distance, brain natriuretic peptide/N-terminal pro-hormone of brain natriuretic peptide, and estimated glomerular filtration rate. RESULTS: The mean age was 32.17 ± 16.3 years. The mean follow-up was 99.41 ± 58.2 months. Thirty-two patients died during follow-up period. Most patients were Eisenmenger syndrome (31%) and simple defects (29.4%). Most patients received monotherapy (76.2%). Most patients were World Health Organization functional class I-II (66.6%). Both models effectively identified risk in our cohort (P =.0001). Patients achieving 2 or 3 noninva-sive low-risk criteria or low-risk category by Registry to Evaluate Early and Long-term Pulmonary Arterial Hypertension Disease Management Lite 2 at follow-up had a significantly reduced risk of death. Registry to Evaluate Early and Long-term Pulmonary Arterial Hypertension Disease Management Lite 2 approximates noninvasive French model at discriminating among patients based on c-index. Age, high risk by Registry to Evaluate Early and Long-term Pulmonary Arterial Hypertension Disease Management Lite 2, and the presence of 2 or 3 low-risk criteria by noninvasive French model emerged as an independent predictors of mortality (multivariate hazard ratio: 1.031, 95% CI: 1.005-1.058, P =.02; hazard ratio: 4.258, CI: 1.143-15.860, P =.031; hazard ratio: 0.095, CI: 0.013-0.672, P =.018, respectively). CONCLUSIONS: Both abbreviated risk assessment tools may provide a simplified and robust method of risk assessment for congenital heart disease-associated pulmonary arterial hypertension. Patients not achieving low risk at follow-up may benefit from aggressive use of available therapies.

Pulmonary arterial hypertension associated with congenital heart disease: lessons learnt from the large Turkish Nationwide Registry (THALES).

Pulmonary hypertension is a group of diseases, including pulmonary arterial hypertension associated with congenital heart disease (APAH-CHD), characterized by progressive deterioration in pulmonary hemodynamics associated with substantial morbidity and mortality risk. THALES is a national multicenter, prospective observational registry, providing data on patients with APAH-CHD. The study comprised APAH-CHD patients (>3 months of age) with confirmed diagnosis of right heart catheterization or echocardiographic findings. Initial and follow-up data were collected via regular hospital visits. Descriptive statistics are used for definitive purposes. Overall, 1034 patients aged 3 months-79 years (median 11.2 [Q1-Q3: 2.2-24.3] years) with APAH-CHD were enrolled at 61 centers, 50.3% being retrospectively enrolled. Most had either Eisenmenger's syndrome (49.2%) or systemic-to-pulmonary shunts (42.7%). Patients were mostly in functional class I-II at the time of diagnosis (46.6%). Mean 6-min walk distance (6MWD) was 369 ± 120 m. Mean pulmonary arterial pressure was 54.7 ± 22.2 mmHg for the whole group, and was highest in patients with Eisenmenger's syndrome. Targeted therapies were noted in 398 (38.5%) patients (monotherapy in 80.4%). Follow-up data were available in 506 patients. Survival at 140 months was 79% and was associated with baseline 6MWD >440 m (p = 0.009), brain natriuretic peptide level < 300 ng/L (p < 0.001). Follow-up 6MWD >165 m (p < 0.0001), brain natriuretic peptide level <300 ng/L (p = 0.031), and targeted therapies (p = 0.004) were also predictive of survival. THALES is the largest registry dedicated to APAH-CHD to date and provides important contributions on demographics, clinical characteristics, and gaps in disease management.

[Turkish Cardiology Association Consensus Report: COVID-19 Pandemic and Cardiovascular Diseases (May 13, 2020)]. / Türk Kardiyoloji Dernegi Uzlasi Raporu: COVID-19 Pandemisi ve Kardiyovasküler Hastaliklar Konusunda Bilinmesi Gerekenler (13 Mayis 2020).

In December 2019, in the city of Wuhan, in the Hubei province of China, treatment-resistant cases of pneumonia emerged and spread rapidly for reasons unknown. A new strain of coronavirus (severe acute respiratory syndrome coronavirus-2 [SARS-CoV-2]) was identified and caused the first pandemic of the 21st century. The virus was officially detected in our country on March 11, 2020, and the number of cases increased rapidly; the virus was isolated in 670 patients within 10 days. The rapid increase in the number of patients has required our physicians to learn to protect both the public and themselves when treating patients with this highly infectious disease. The group most affected by the outbreak and with the highest mortality rate is elderly patients with known cardiovascular disease. Therefore, it is necessary for cardiology specialists to take an active role in combating the epidemic. The aim of this article is to make a brief assessment of current information regarding the management of cardiovascular patients affected by COVID-19 and to provide practical suggestions to cardiology specialists about problems and questions they have frequently encountered.

[Consensus Report from Turkish Society of Cardiology: COVID-19 and Cardiovascular Diseases. What cardiologists should know. (25th March 2020)]. / Türk Kardiyoloji Dernegi Uzlasi Raporu: COVID-19 Pandemisi ve Kardiyovasküler Hastaliklar Konusunda Bilinmesi Gerekenler (25 Mart 2020).

In December 2019, in the city of Wuhan, in the Hubei province of China, treatment-resistant cases of pneumonia emerged and spread rapidly for reasons unknown. A new strain of coronavirus (severe acute respiratory syndrome coronavirus-2 [SARS-CoV-2]) was identified and caused the first pandemic of the 21st century. The virus was officially detected in our country on March 11, 2020, and the number of cases increased rapidly; the virus was isolated in 670 patients within 10 days. The rapid increase in the number of patients has required our physicians to learn to protect both the public and themselves when treating patients with this highly infectious disease. The group most affected by the outbreak and with the highest mortality rate is elderly patients with known cardiovascular disease. Therefore, it is necessary for cardiology specialists to take an active role in combating the epidemic. The aim of this article is to make a brief assessment of current information regarding the management of cardiovascular patients affected by COVID-19 and to provide practical suggestions to cardiology specialists about problems and questions they have frequently encountered.

Do Restoring and Maintaining Sinus Rhythm Have a Beneficial Effect on Secondary Prevention of Stroke in Patients With Atrial Fibrillation? A Pilot Study.

We aimed to determine whether attempts to restore and maintain sinus rhythm will reduce recurrent stroke in patients with atrial fibrillation (AF). Patients (n = 245) between March 1998 and May 2002 with AF who had an ischemic stroke including transient ischemic attack 1 to 12 months before transesophageal echocardiographic examination and had been followed for 3 years were retrospectively reviewed. Cardioversion was attempted in 130 patients; 117 (90%) patients were successfully cardioverted (rhythm control group). The 13 patients who could not be cardioverted and 115 patients who did not undergo cardioversion were assigned to the rate control group. Age, gender, ischemic heart disease, hypertension, diabetes mellitus, congestive heart failure, mitral valve disease, and left atrial diameter were similar in both groups. The rhythm control group included 56 patients (48.7%) who were still in sinus rhythm after 3 years. During follow-up, there were 2 embolic events (3.4%) and 2 deaths (3.4%) in the rhythm control group, whereas 18 embolic events (14.6%) and 18 deaths (14.6%) occurred in the rate control group (P = .049 and P = .049, respectively). Restoration and maintenance of sinus rhythm seems to have a beneficial effect on secondary prevention of stroke in patients with AF.

[Pulmonary hypertension in the elderly]. / Yasli hastalarda pulmoner hipertansiyon.

Pulmonary hypertension is defined as 25mmHg or higher mean pulmonary arterial pressure measured by right heart catheterization. Miscellaneous diseases may cause PH. Among these are pulmonary arterial hypertension (PAH), left heart disease, pulmonary disorders and hypoemia, and chronic thromboembolic disease. Pulmonary hypertension (PH) is seen frequently in the elderly. Although diseases resulting in systolic and diastolic dysfunction with accompanying pulmonary venous hypertension (PVH) account for a large percentage of PH in this population, PH associated with other respiratory diseases, such as chronic obstructive pulmonary disease (COPD), interstitial lung disease (ILD), and sleep apnea can also be seen. Idiopathic pulmonary arterial hypertension (IPAH) is, although rarely, seen and requires careful exclusion in older patients. PAH associated with connective tissue disease is the most encountered form of PAH. Since the treatment to all these disease is different, differential diagnosis has the utmost importance.

Four-Dimensional Speckle Tracking Echocardiography in Patients with Hypertrophic Cardiomyopathy.

OBJECTIVE: Abnormal left ventricular (LV) deformational mechanics have been demonstrated in patients with hypertrophic cardiomyopathy (HCM) using two-dimensional (2D) speckle tracking echocardiography, but there is not enough information about the four-dimensional speckle tracking echocardiography (4DSTE) in these patients. The objective of the study was to identify and quantify the left ventricular contractility in patients with HCM using 4DSTE. METHODS: Thirty patients [age, 54.6 ± 12.1 years; 12 (40%) women] with diagnosis of nonobstructive HCM and 20 healthy controls [age, 47.42 ± 11.43 years; 8 (40%) women] underwent 4DSTE measurement of longitudinal, radial, circumferential, and area strains. RESULTS: Patients with HCM showed lower longitudinal (-13.5% vs. -20.3%, P < 0.001) and radial (33.4% vs. 43.6%, P < 0.001) strain, but higher circumferential (-22.7% vs. -15.9%, P < 0.001) and area (-30.7% vs. -22.1%, P < 0.001) strain than control subjects. Peak LV twist showed significantly higher values in patients with HCM (13.7 ± 5.3 vs. 11.3 ± 4.8, P < 0.005). CONCLUSIONS: Patients with HCM have reduced longitudinal and radial strain but increased circumferential and area strain on 4DSTE. These results are found appropriate with previous 2DSTE studies. Thus, 4DSTE is useful to determine LV deformational mechanics.

A Case of Isolated Cardiac Hydatid Cyst that Mimics Lymphoproliferative Malignancy.

Cardiac cystic echinococcosis is a rare parasitic infestation caused by Echinococcus granulosus larvae and it composes 0.5-2% of all human cystic echinococcosis cases. The left ventricle is the most common affected area followed by right ventricle, interventricular septum, left atrium, right atrium, and interatrial septum. The diagnosis is difficult because of nonspecific clinical and radiographic findings. We present a case of isolated apical cardiac cystic echinococcosis mimicking lymphoproliferative disease.

Factors predicting long-term mortality in patients with hypertrophic cardiomyopathy.

OBJECTIVES: In this study, we aimed to elucidate the factors affecting long-term all-cause mortality in patients with hypertrophic cardiomyopathy (HCM). METHOD: We retrospectively examined 31 patients (22 males and 9 females) diagnosed with HCM from 1999 to 2013. All subjects had sinus rhythm at the time of evaluation. Four patients had history of paroxysmal atrial fibrillation (PAF). In addition to echocardiographic examination plasma angiotensin-converting enzyme (ACE) activity and gene polymorphism were determined. The variables that were found to be significant in mortality were then included in multivariate analysis. RESULTS: At the final follow-up examination, 12 patients had died, including 2 due to congestive heart failure and 10 due to sudden cardiac death. Patients with PAF had significantly higher mortality (P = 0.008). Moreover, left ventricular (LV) end-diastolic diameter (P = 0.04), LV systolic diameter (P = 0.001), LV mass index (P = 0.01), and left atrium diameter (P = 0.003) were found to be significantly correlated with mortality. However, no significant correlation was noted between mortality and age, type of HCM (familial/nonfamilial and obstructive/nonobstructive), ACE gene polymorphism, and plasma ACE level. In the multivariate analysis, left atrial (LA) diameter was still significantly associated with mortality. The LA diameter with a cutoff value of 4.1 cm predicted 13-year mortality with a sensitivity of 82% and specificity of 78%. CONCLUSION: Instead of the ACE genotype and activity, echocardiographic evaluation findings such as LV systolic and diastolic diameters, LV mass index, and particularly LA dimension may predict long-term mortality in patients with HCM. PAF has also significant importance in the long-term mortality in patients with HCM.

Long-term prognosis of mild functional tricuspid regurgitation after mitral valve replacement.

OBJECTIVE: Functional tricuspid regurgitation (FTR) is the most common type of tricuspid insufficiency and occurs approximately in 30% of patients with mitral valve disease. The major etiologic factor in the triggering of right ventricular dilation and thus causing functional tricuspid regurgitation, is pulmonary artery hypertension secondary to mitral valve disease. We aimed to analyze long-term outcomes of patients with mild tricuspid regurgitation at the time of mitral valve replacement. METHODS: Sixty-six patients with mild tricuspid insufficiency who underwent mitral valve replacement were included in this observational retrospective study. Mean follow-up time was 8.3 ± 0.7 years. Patients whose tricuspid regurgitation remained unchanged or decreased following operation were enrolled to group 1 (n=32), patients whose tricuspid regurgitation increased were included to group 2 (n=34) and data were compared statistically with t-test, Mann-Whitney U, Chi-square and Fisher Exact test. Multiple regression analysis was performed to determine independent risk factors for FTR progression. REESULTS:Preoperatively female gender (p=0.02), body surface area (p=0.04), left atrium diameter (p=0.01), functional capacity (p=0.03), right ventricle diameter (p=0.04), and left ventricle mass index (p=0.04) were found to be statistically significant between groups. In the follow-up; functional capacity, grade of tricuspid insufficiency, pulmonary artery pressure, vena contracta width (p<0.001), TAPSE (tricuspid annular plane systolic excursion index) (p=0.04), annulus diameter (p=0.02), right ventricle diameter (p=0.01), left ventricle mass index (p=0.05), and ejection fraction (p=0.02) were found to be statistically different between groups. In multiple logistic regression analysis; preoperative LA diameter (OR=5.05; 95% CI:1.49-17.12; p=0.009) and female gender (OR=10.93; 95% CI:1.77-67.31; p=0.01) were found as independent risk factors for FTR progression. CONCLUSION: This study revealed that mild FTR might advance to moderate to severe grade in more than half of the patients in the follow-up. Thus, surgical approach to even mild FTR should be individualized based on patient's risk assessment.

P-wave dispersion and its relationship with the severity of the disease in patients with stable coronary artery disease.

OBJECTIVE: P- wave dispersion (PD) is an indicator of inhomogeneous and discontinuous propagation of sinus impulses. In the present study we aimed to investigate the PD and its association with the severity of the disease. in patients with stable coronary artery disease. METHODS: We prospectively analyzed 60 subjects with coronary artery disease (CAD) and 25 subjects with nor-mal coronary angiograms (control group). The maximum and minimum P-wave duration and PD were measured from the 12-lead surface electrocardiograms. The CAD severity was assessed by the severity score (Gensini score) and the number of vessels involved (vessel score). RESULTS: P max was longer in CAD group compared with the control group (p<0.001). PD was greater in the CAD group, compared with the control group (p<0.001). However, P min did not differ between the two groups. In bi-variate correlation, increased PD was correlated with presence of diabetes mellitus (r=0.316, p=0.014), smoking (r=0.348, p=0.006), left ventricular ejection fraction (r=-0.372, p=0.003), vessel score (r=0.848, p=0.001), and Gensini score (r=0.825, p=0.001). Multiple linear regression analysis showed that PD was independently associ¬ated with vessel score ((3=0.139, p=0.002) and Gensini score ((3=0.132, p=0.007). CONCLUSION: PD was greater in patients with CAD than in controls and it was associated with CAD severity.

Value of stress echocardiography in mitral stenosis.

Mitral stenosis (MS) is a common disease that causes substantial morbidity. Timely recognition and treatment of the mitral stenotic lesion is very important. Stress echocardiography plays an important role in evaluating asymptomatic patients with significant mitral stenosis and symptomatic patients with only mild disease at rest. In patients with mitral stenosis, stress echocardiography protocols assess for the change in the mean transmitral pressure gradient and a peak pulmonary artery systolic pressure during exercise. In this review, clinical use of stress echocardiography in patients with mitral stenosis is summarized.

Matrix metalloproteinase-2 and -9 levels in patients with dilated ascending aorta and bicuspid aortic valve.

BACKGROUND: Predictors of aortic dilatation are not well-described in patients with bicuspid aortic valve (BAV). Changes in extracellular matrix composition in the aortic wall may play an important role. Our study aimed to examine the relationship between ascending aortic dilatation and biochemical markers for collagen metabolism, such as matrix metalloproteinase-2 (MMP-2) and matrix metalloproteinase-9 (MMP-9) levels in patients with BAV. METHODS: All patients underwent cardiac echocardiography using a standard protocol, and aortic measurements were made in end-diastole. One hundred twelve BAV patients with no or mild valvular impairment were recruited and grouped according to the aortic dimensions corrected for body surface area (BSA) and age. There were 54 patients with dilated ascending aorta (Group 1) and 58 patients with nondilated ascending aorta (group 2). The plasma levels of MMP-2 and MMP-9 were determined by ELISA. RESULTS: The mean ascending aorta diameter was 4.49 ± 0.49 mm in group 1 and 3.51 ± 0.46 mm in group 2 (P < 0.001). There were no significant difference in gender, BSA, presence of hypertension, diabetes mellitus, hyperlipidemia, and smoking between the 2 groups. Nevertheless, no significant difference was observed in the levels of MMP-2 and MMP-9 between the 2 groups. The ascending aorta diameter correlated significantly with age (r = 0.438 P < 0.001). No significant correlation was observed between plasma MMP-2 and MMP-9 concentration and ascending aorta diameter, respectively (r = -0.005 P = 0.58, r = -0.106 P = 0.07). Multivariate analysis showed that age was independent predictor of aortic dilatation (P ≤ 0.001). CONCLUSION: Age was an independent predictor of aortic dilatation in patients with BAV, whereas MMP-2 and 9 levels were not relevant by aortic dilatation.

Dilatation of the ascending aorta and serum alpha 1-antitrypsin level in patients with bicuspid aortic valve.

Predictors of aortic dilatation are not well described in patients with bicuspid aortic valves (BAV). This study sought to examine the relationship between proximal aortic dilatation and matrix metalloproteinase-9 (MMP-9) and alpha 1-antitrypsin (α1AT) levels in patients with BAV. All patients underwent echocardiography using a standard protocol, and aortic measurements were taken in end-diastole. We studied 82 patients with BAV and categorized them into two groups according to aortic dimensions corrected for body surface area and age. The plasma level of α1AT was routinely determined using a BN ProSpec analyzer (Siemens Healthcare Diagnostics, Marburg, Germany), and that of MMP-9 were determined by ELISA (RayBiotech Inc. Norcross, GA, USA). Statistical analysis was performed using the Statistical Package for Social Sciences (SPSS; SPSS Inc., Chicago, IL, USA) software for Windows version 12. This study included patients with BAV with no or mild valvular impairment. There were no significant differences between groups in terms of gender, body surface area, associated hypertension, diabetes mellitus, hyperlipidemia, or smoking. The mean ascending aortic diameter was 4.38 ± 0.5 mm in group 1 and 3.34 ± 0.35 mm in group 2 (p < 0.001). Plasma concentration of α1AT in patients with ascending aortic dilatation was significantly lower than that in the non-dilated group (1.32 ± 0.27 and 1.49 ± 0.25 g/l, respectively; p = 0.005). However, no significant difference was found in the MMP-9 level between the two groups (336.49 ± 233.11 and 336.39 ± 268.072 pg/ml, respectively; p = 0.96). We observed a significantly negative correlation between ascending aortic diameter and α1AT level (r = -0.300, p = 0.006) and a positive correlation between ascending aortic diameter and age (r = 0.413, p < 0.001). No significant correlation was found between plasma MMP-9 concentration and ascending aortic diameter (r = -0.008, p = 0.94). A multiple linear regression analysis was performed, including age, α1AT level, MMP-9 level, and left ventricular diastolic diameter. In this analysis, α1AT level and age were the independent predictors of aortic dilatation (p = 0.03 and p = 0.02, respectively).

Large cardiac hydatid cyst in the interventricular septum.

Cystic hydatid disease results from infection with the larval or adult form of the Echinococcus granulosus tapeworm. Cardiac involvement is seen in 0.5% to 2% of patients with hydatid disease, and involvement of the interventricular septum is even rarer. Herein, we report our surgical treatment of a large cardiac hydatid cyst in the interventricular septum. A 39-year-old woman presented with dyspnea. Transthoracic echocardiography revealed a large cyst in the apical part of the interventricular septum. Thoracic computed tomography showed a cystic lesion in that site, and magnetic resonance imaging confirmed the presence of a 50 × 55-mm mass. The patient was placed on cardiopulmonary bypass. Hypertonic saline solution-soaked sponges were distributed within the pericardial cavity to prevent local invasion of the parasite intraoperatively. Through an incision parallel to the left anterior descending coronary artery, and without opening adjacent cardiac chambers, we aspirated the entire contents of the cyst, removed its germinative membrane, and washed the cavity with 20% hypertonic saline solution. The patient recovered uneventfully. She had begun taking albendazole 5 days preoperatively, and this therapy was continued for 12 weeks postoperatively. In cases of an interventricular cardiac hydatid cyst, the combination of surgical resection, washout of the remaining cavity with hypertonic saline solution, and albendazole therapy typically yields excellent results.