The Outcome of Patients with Pulmonary Arterial Hypertension and Chronic Thromboembolic Pulmonary Hypertension during the COVID-19 Pandemic.

BACKGROUND: The coronavirus 2019 (COVID-19) has affected the lives of many people worldwide. Patients with chronic underlying morbidities are vulnerable to get the severe form of the infection. The goal of this study was to evaluate the outcome of patients with pulmonary arterial hypertension during the COVID-19 pandemic in Iran. METHODS: This cross-sectional study was conducted at a large tertiary center for pulmonary artery hypertension (PAH) patients. The primary end point was the prevalence of SARS-CoV-2 infection in PAH patients. The secondary end points were investigating the severity and mortality of COVID-19 infection in PAH patients during the COVID-19 pandemic. RESULTS: Totally 75 patients were enrolled in the study from December 2019 to October 2021 and 64% were female. The mean ± SD age was 49 ± 16 years. The prevalence of COVID-19 in PAH/chronic thromboembolic pulmonary hypertension patients was 44%. About 66.7% of patients had comorbidities, which was a prognostic factor for COVID-19 infection in PAH patients (P < 0.001). Fifty-six percent of infected patients were asymptomatic. The most reported symptoms in symptomatic patients were fever (28%) and malaise (29%). Twelve percent of patients were admitted with severe symptoms. The mortality rate in infected individuals was 3.7%. CONCLUSIONS: COVID-19 infection in PAH/chronic thromboembolic pulmonary hypertension patients seems to be associated with high mortality and morbidity. More scientific proof is needed to clarify different aspect of COVID-19 infection in this population.

High cardiac output state due to pelvic arterio-venous shunt: a case report and review of the literature.

We are presenting a 35-year-old woman with past medical history of disseminated leiomyomatosis who presented with heart failure symptoms and was found to have post-capillary pulmonary hypertension and high cardiac output state in right heart catheterization secondary to a huge pelvic arterio-venous fistula.

Relationship between Complete Revascularization and Survival after Post-Infarction Ventricular Septal Rupture.

INTRODUCTION: A well-known and fatal complication of myocardial infarction (MI) is post-infarction ventricular septal rupture (VSR). The benefits and risks associated with coronary angiography and subsequent coronary artery bypass grafting in these patients have sparked controversy. The aim of this study was to determine the outcome of revascularization following MI. METHOD: Patients aged between 55 and 78 years were considered for the post-infarction ventricular septal rupture from 2011 to 2017. Factors such as age, sex, anthropometric measurements, systolic and diastolic blood pressure (SBP and DBP), and biochemical parameters like CPK-MB, cholesterol, low-density lipoprotein, high-density lipoprotein, and triglycerides were measured using standard methods.The estimated Glomerular Filtration Rate (eGFR), a measure of kidney function, was also determined. Additionally, coronary angiographic factors including ECG changes, left ventricular (LV) systolic function, right ventricular (RV) function, Pulmonary Artery Pressure (PAP), proximal coronary lesions in VSR, systolic PAP, Right Atrial Pressure (RAP), and mortality rate were determined. RESULTS: The study enrolled a total of 81 patients who had been surgically treated for post-infarction VSR. These patients were divided into two groups: survivors (n=35) and non-survivors (N=41). The mean systolic and diastolic blood pressure was higher in the survivor group (115.3 ± 18.7 vs. 96.3 ± 25.3 and 74.6 ± 12.2 vs. 61.2 ± 19.0, P=0.001). PCI was performed in 2.9% of survivors and 9.8% of non-survivors. Angiographic data revealed that 17 (33%) and 33 (63%) patients had single and multiple coronary artery diseases, respectively. CPK-MB levels were significantly higher in the non-survivors group (P<0.05). Echocardiographic findings, including LV ejection fraction, RV ejection fraction, systolic PAP, and the anatomic location of VSR, did not significantly differ between survivors and non-survivors. CONCLUSION: Based on these findings, it is recommended to avoid complete revascularization during surgical repair of post-infarction ventricular septal rupture, as it would not improve the outcome.

Myocarditis following rAd26 and rAd5 vector-based COVID-19 vaccine: case report.

SARS-CoV-2 vaccines provide a safe solution with a major impact on reducing the spread of the virus and mild side effects. Research has shown rare cases of myocarditis after mRNA vaccines. This study presents a 29-year-old male with chest pain after 48 h of receiving rAd26 and rAd5 vector-based COVID-19 vaccine (Sputnik V vaccine). The electrocardiogram revealed ST-segment elevation. Also, the laboratory screening was remarkable for elevated cardiac Troponin-I level, and leukocytosis; and echocardiography depicted severe left ventricular systolic dysfunction. Overall, endomyocardial biopsy proved lymphocytic myocarditis such that the patient was successfully treated with immunosuppressive and guideline-directed medical treatment.

Remdesivir associated sinus bradycardia in patients with COVID-19: A prospective longitudinal study.

Background: Remdesivir is effective against SARS-Cov-2 with little evidence of its adverse effect on the cardiac system. The aim of the present study is investigating the incidence of bradycardia in COVID-19 patients treated with Remdesivir. Methods: This prospective longitudinal study was conducted in a tertiary center on COVID-19 patients for Remdesivir therapy. The objectives were to investigate the incidence of sinus bradycardia, and also the association between their demographics, underlying diseases, and the disease severity with developing bradycardia in COVID-19 patients treated with Remdesivir. Results: Of 177 patients, 44% were male. The mean (±standard deviation) age of patients was 49.79 ± 15.16 years old. Also, 33% were hospitalized due to more severe symptoms. Oxygen support was required for all hospitalized subjects. A total of 40% of the patients had comorbidities, with the most common comorbidity being hypertension. The overall incidence of bradycardia (heart rate<60 bpm) in patients receiving Remdesivir was 27%, of whom 70% had extreme bradycardia (heart rate <50 bpm). There was also a statistically significant reduction in heart rate after five doses of Remdesivir compared to the baseline heart rates. In the multivariable model, none of the covariates including age above 60 years, female sex, CRP>50 mg/L, O2 saturation<90%, underlying cardiovascular disease, hypertension and diabetes mellitus, and beta-blockers were associated with Remdesivir-induced bradycardia. No association was found between the COVID-19 severity indicators and bradycardia. Conclusion: As sinus bradycardia is a prevalent adverse cardiac effect of Remdesivir, it is recommended that all COVID-19 patients receiving Remdesivir, be evaluated for heart rate based on examination; and in the case of bradyarrhythmia, cardiac monitoring should be performed during administration to prevent adverse drug reactions.

Whole-Exome Sequencing Identified a Novel Variant (C.405_422+39del) in DSP Gene in an Iranian Pedigree with Familial Dilated Cardiomyopathy.

BACKGROUND: Dilated cardiomyopathy (DCM) is a progressive heart condition characterized by left ventricular chamber enlargement associated with systolic heart failure and prolonged action potential duration. Genetic variations in genes that encode cytoskeleton, sarcomere, and nuclear envelope proteins are responsible for 45% of cases. In our study, we focused on a pedigree with familial DCM to decipher the potential genetic cause(s) in affected members developing arrhythmia, end-stage heart failure, and sudden death. METHODS: Whole-exome sequencing (WES) was exploited for a 27-year-old heart-transplanted female as the proband, and the derived data were filtered using the standard pipelines. RESULTS: A 57-nucleotide deletion (c.405_422+39del) in the desmoplakin gene (DSP) (NM_004415.4) was identified as a novel pathogenic variant. Familial segregation analysis indicated that this variant is present in clinically affected members and absent in unaffected members. CONCLUSION: It seems that the detected variant induces intron retention, resulting in a premature stop codon in intron 3 of DSP leading to production of a truncated, nonfunctional protein. Additionally, it can trigger a nonsense-mediated mRNA decay pathway associated with inhibition of protein production. The present study results illustrated that a novel deletion in DSP can cause DCM in an Iranian family.

A simple hemodynamic parameter to predict clinical worsening in pulmonary arterial hypertension.

BACKGROUND: Predicting prognosis is a cornerstone in management of pulmonary arterial hypertension. Hemodynamic parameters are among the robust indicators of right ventricular function and prognosis. In this study we have investigated the association of a simple hemodynamic parameter with clinical worsening in pulmonary arterial hypertension. METHODS AND PATIENTS: 120 patients were enrolled in a single center prospective cohort study after confirmation of precapillary pulmonary hypertension and were followed for an average of 36months on guideline recommended treatment protocols. cSvO2 was calculated as the ratio of right atrial pressure over Mixed Venous Oxygen Saturation. Independent predictors of clinical worsening were identified using multivariable Cox regression models. RESULTS: By the end of the follow up a total of 21 patients died and 63 were hospitalized for pulmonary hypertension. Time-to-event Cox regression model showed a strong association between cSvO2 and time to clinical worsening (HR: 250.13, CI: (38.56-1622.34) &p-value: <0.0001). CONCLUSION: The index of cSvO2 includes both parameters of cardiac output and right ventricular filling pressure and might be beneficial in predicting clinical worsening in patients with pulmonary arterial hypertension.

Lower Doses of Bosentan in Combination With Sildenafil Might be Beneficial in Pulmonary Arterial Hypertension.

BACKGROUND: Endothelin-receptor-antagonist, bosentan, has been found to improve the functional capacity and cardiopulmonary hemodynamics in Pulmonary Arterial Hypertension (PAH). Clinical trials have shown the preferable dosage of 125 mg, twice daily, regarding both efficacy and safety. OBJECTIVES: The purpose of this study was to investigate the effects of lower doses of bosentan (62.5 mg, twice daily) in combination with sildenafil on exercise capacity and clinical events, in 41 patients with idiopathic pulmonary hypertension or chronic thromboembolic pulmonary hypertension (CTEPH). PATIENTS AND METHODS: We assigned 41 patients with PAH (non-reactive idiopathic or non-operable chronic thromboembolic) to receive 62.5 mg of bosentan twice daily as combination therapy and evaluated the New York heart association (NYHA) functional class, 6-minutes-walk-distance (6MWD), time to clinical worsening, echocardiographic indexes and clinical events, for an average of 18.5 ± 9.5 months. RESULTS: No adverse drug reaction was observed during the follow-up. Clinical worsening occurred in six (14%) patients, at least one year after treatment, two of the cases failed to respond to 125 mg, twice daily and died. Eight (19%) remained in FC I_II, but didn't reach the goal of 380 meters for 6MWD. All other patients reached the treatment goals according to the latest European society of cardiology (ESC) guidelines. CONCLUSIONS: We observed acceptable results regarding both efficacy and safety with 62.5 mg of bosentan, twice daily in this group of patients. Further clinical trials investigating PAH with lower dosages of bosentan may be warranted.