Fatal Large Intracardiac in Situ Thrombus in a Mildly Symptomatic COVID-19 Patient: A Case Report.

Background: The coronavirus disease 2019 (COVID-19) has caused a global pandemic with a significant negative impact on health worldwide. Although it primarily involves the respiratory tract system, cardiovascular complications, particularly thrombotic events are increasingly reported and are associated with adverse outcomes. Case Summary: We describe a 30-year-old male who presented with exaggerated dyspnea and hypoxia with mild constitutional symptoms along with a positive PCR test for SARS-CoV-2. He was a known case of bronchiectasis and large bullae in the left lung. Transthoracic echocardiography showed a large right ventricle (RV) mass entrapped within RV papillary muscles and apex. Cardiac MRI was suggestive of RV thrombosis. Regarding diffuse underlying lung disease and improving respiratory symptoms with COVID-19 negative PCR test, we decided to prescribe anticoagulation with heparin infusion and then oral anticoagulant with Rivaroxaban 20 mg daily and follow up after 30 days. Unfortunately, the patient died after 2 weeks in his hometown. Conclusion: Thrombotic complications are frequently encountered in COVID-19 patients. We report a fatal case of large intracardiac thrombus associated with SARS-CoV-2 infection managed with anticoagulant therapy with a catastrophic outcome.

Clinical evaluation of nebulized verapamil in out-patients with pulmonary hypertension secondary to chronic obstructive pulmonary disease.

OBJECTIVE: Chronic obstructive pulmonary disease (COPD) is associated with many health complications, including pulmonary hypertension (PH). Although oral calcium channel blockers have shown promising results in managing COPD-induced PH, significant systemic side effects may limit their use in this population. Administering verapamil through nebulization can be an alternative approach. We aim to assess the possible therapeutic effects of verapamil inhalation in out-patients with pulmonary hypertension (PH) secondary to COPD. METHODS: A double-blind, randomized placebo-controlled clinical trial was conducted. Patients with PH were randomly assigned to two groups of 15 participants. The intervention group received a short-term single dose of 10 mg nebulized verapamil (4 ampoules of 2.5 mg/ml verapamil solutions). The control group received nebulized distilled water as a placebo in addition to their standard treatment throughout the study. RESULTS: Systolic pulmonary artery pressure (sPAP) did not improve as a primary outcome significantly in patients receiving nebulized verapamil compared with those on placebo (p = 0.89). Spirometry results showed a significant improvement in FVC in the intervention group from 1.72 ± 0.63 to 1.85 ± 0.58 L (p = 0.00), and FEV1/FVC ratio decreased significantly after verapamil administration (p = 0.027). CONCLUSION: Verapamil did not improve any of the pulmonary artery or RV parameters in patients with COPD-associated, but it did improve SpO2 and increase FVC, which revealed us possibility of verapamil in treating V/Q mismatch. The improved gas exchange may have been due to improvements in FVC as reflected in the improved spirometry. Higher doses of verapamil may be more efficacious and can be the subject of future trials.

A novel mutation in PRKAR1A gene in a patient with Carney complex presenting with pituitary macroadenoma, acromegaly, Cushing's syndrome and recurrent atrial myxoma

SUMMARY Carney complex (CNC) is a rare syndrome of multiple endocrine and non-endocrine tumors. In this paper we present a 23-year-old Iranian woman with CNC who harbored a novel mutation (c.642dupT) in PRKAR1A gene. This patient presented with pituitary macroadenoma, acromegaly, recurrent atrial myxoma, Cushing's syndrome secondary to primary pigmented nodular adrenocortical disease and pigmented schwanoma of the skin. PRKAR1A gene was PCR amplified using genomic DNA and analyzed for sequence variants which revealed the novel mutation resulting in substitution of amino acid cysteine instead of the naturally occurring valine in the peptide chain and a premature stop codon at position 18 (V215CfsX18). This change leads to development of tumors in different organs due to lack of tumor suppressive activity secondary to failure of synthesis of the related protein.

A novel mutation in PRKAR1A gene in a patient with Carney complex presenting with pituitary macroadenoma, acromegaly, Cushing's syndrome and recurrent atrial myxoma.

Carney complex (CNC) is a rare syndrome of multiple endocrine and non-endocrine tumors. In this paper we present a 23-year-old Iranian woman with CNC who harbored a novel mutation (c.642dupT) in PRKAR1A gene. This patient presented with pituitary macroadenoma, acromegaly, recurrent atrial myxoma, Cushing's syndrome secondary to primary pigmented nodular adrenocortical disease and pigmented schwanoma of the skin. PRKAR1A gene was PCR amplified using genomic DNA and analyzed for sequence variants which revealed the novel mutation resulting in substitution of amino acid cysteine instead of the naturally occurring valine in the peptide chain and a premature stop codon at position 18 (V215CfsX18). This change leads to development of tumors in different organs due to lack of tumor suppressive activity secondary to failure of synthesis of the related protein.

Evaluation of cardiomyopathy diagnosis in heart transplant recipients: comparison of echocardiographic and pathologic classification.

BACKGROUND: Definite diagnosis of cardiomyopathy types can be challenging in end-stage disease process. New growing data have suggested that there is inconsistency between echocardiography and pathology in defining type of cardiomyopathy before and after heart transplantation. The aim of the present study was to compare the pre-heart transplant echocardiographic diagnosis of cardiomyopathy with the results of post-transplant pathologic diagnosis. RESULTS: In this retrospective cross-sectional clinicopathological study, 100 consecutive patients have undergone heart transplantation in Masih-Daneshvari hospital, Tehran, Iran, between 2010 and 2019. The mean age of patients was 40 ± 13 years and 79% of patients were male. The frequency of different types of cardiomyopathy was significantly different between two diagnostic tools (echocardiography versus pathology, P < 0.001). On the other hand, in 24 patients, the results of echocardiography as regard to the type of cardiomyopathy were inconsistent with pathologic findings. CONCLUSION: Based on the findings of the present study, it could be concluded that there is a significant difference between echocardiographic and pathologic diagnosis of cardiomyopathy; therefore, it is necessary to use additional tools for definite diagnosis of cardiomyopathy like advanced cardiac imaging or even endomyocardial biopsy before heart transplantation to reach an appropriate treatment strategy.

Superior vena cava obstruction and pulmonary artery involvement as rare findings of immunoglobulin G4-related disease.

We describe a case of immunoglobulin G4-related lung disease presenting as chronic pulmonary hypertension with involvement of right pulmonary artery and superior vena cava. Immunoglobulin G4- related disease is a rare systemic sclerosing disease with autoimmune entity that causes fibrotic, often mass-like manifestations that variably affect different organ systems and can be mistaken with other disorders. Timely diagnosis requires awareness on the part of clinicians and pathologists as well as radiologists to the variable manifestations of this newly recognized disorder. .

Curable Syncope in Primary Pulmonary Hypertension with Novel Atrial Flow Regulator.

Pulmonary artery hypertension (PAH) occurs when mean pulmonary artery pressure (PAP) is higher than 25 mmHg in rest or 30 mmHg during activity. Idiopathic pulmonary artery hypertension (IPAH) is defined as PAH without a definite reason. The IPAH is a rare disease with a high mortality rate if left untreated. To date, there has been no definite cure for this entity, and most treatments are for symptom relief or improvement in the quality of life. For many years, decompressing the right heart through a hole in the interatrial septum has been advised to alleviate some of their symptoms, specifically syncope. Atrial flow regulator (AFR) is a device to make an iatrogenic interatrial hole and finally a unidirectional shunt. It has been used for some PAH patients for symptom relief. Herein, we report a 36-year-old female case with diagnosed IPAH for 6 years. In the last 3 years, the case had frequent syncope with shortening intervals. The AFR was implanted for her without any complications. Then, within 6 months of follow-up, she had only one syncope episode. A significant change was observed in her 6-minute walk and PAP.

Removing an Entrapped Pigtail Catheter by Re-enforcing a Traditional Method.

Right heart catheterization is the main step in the evaluation of pulmonary hypertension including Chronic Thromboembolic Pulmonary Hypertension (CTEPH) and is considered a relatively safe procedure. Complications can occur including perforation, tamponade, bleeding, etc. requiring different types of interventions such as manipulation or surgery. Here, we have described a case of pigtail catheter entrapment and the method we used to free it without invasive measures.

Blood Pressure Measurement and Left Ventricular Mass Index in Hemodialysis Patients: Comparison of Several Methods.

INTRODUCTION: Systemic arterial hypertension is prevalent in end-stage renal disease and is closely associated with left ventricular hypertrophy (LVH). Blood pressure (BP) behavior is unique in this population, and it is not clear which BP measurement should be used for treatment guidance. We aimed to evaluate the association of several methods of BP measurement with left ventricular mass index (LVMI) as hypertensive end-organ damage. MATERIALS AND METHODS: Patients on maintenance hemodialysis, 3 or 4 times per week for at least 3 months, were enrolled. We compared the diagnostic value of 6 different methods of BP measurement, including predialysis, postdialysis, interdialysis, and standard BP measurements as well as ambulatory blood pressure monitoring (ABPM) and home blood pressure monitoring, based on LVMI as the gold standard. RESULTS: Twenty patients, including 9 women and 11 men were enrolled. Ten patients (50%) had LVH and the others had normal LVMI (LVMI > 100 g/m2 for women and > 131 100 g/m2 for men). Only predialysis and postdialysis systolic BP values were significantly associated with LVMI (P = .02 and P = .02, respectively). CONCLUSIONS: Predialysis and postdialysis systolic BP values maybe reliable for detecting hypertension in hemodialysis patients, although according to previous data, the importance of self and ambulatory BP monitoring could not be overlooked.

Traumatic Transection of Descending Thoracic Aorta Presenting as Pseudo- Coarctation.

Background: Thoracic Aortic Injury (TAI) due to penetrating or blunt chest trauma is a critical life-threatening aortic injury. Its diagnosis and treatment always is challenging. An 18-year-old male was admitted due to blunt chest trauma after a high-impact road traffic collision. According to presenting dyspnea, an emergency chest-x-ray revealed left hemothorax for which chest tube was inserted. Hemodynamic monitoring demonstrated uncontrolled hypertension with systolic blood pressure of 200-220 mmHg despite multiple anti-hypertensive drugs. Trans-Thoracic Echocardiography (TTE) revealed abnormal Doppler flow pattern in proximal descending thoracic aorta suggestive for probable coarctation of aorta. Chest CT scan revealed pseudoaneurysm of the descending thoracic aorta just below the isthmus. Due to uncontrolled hypertension, persistent hemothorax and probable aortic pseudoaneurysm presenting as aortic luminal narrowing, surgical resection of the aneurysm was planned. The postoperative course was uneventful and blood pressure normalized without any drugs. Patient is normotensive after 8 years follow up.

Asymptomatic Giant Lipoma of the Right Ventricular Outflow Tract: A Case Report.

Cardiac lipomas are extremely rare tumors of the heart. They are usually symptomatic and rarely may be found incidentally in autopsies. Here we describe a 23-year-old healthy man, in whose physical examination for employment a murmur was found incidentally. Transthoracic and then transesophageal echocardiographic examination showed a 4-cm oval-shaped mass in the right ventricular outflow tract. He underwent elective surgery, during which the tumor was removed under cardiopulmonary bypass and aortic-cross clamping via right atriotomy. The postoperative course was uneventful, and the patient was in good condition at 1 year's follow-up.

Relationship between Serum Uric Acid Levels and the Severity of Pulmonary Hypertension.

BACKGROUND: Right heart catheterization is the gold standard test for diagnosis and clinical assessment of the patients with pulmonary hypertension (PH). In recent years, the usefulness of cheaper and non-invasive tests in the follow-up of PH patients is being studied. The aim of the present study was to evaluate the relationship between serum uric acid level and severity of pulmonary hypertension in PH patients. MATERIALS AND METHODS: In a cross-sectional study, serum uric acid was measured in 110 patients with PH (63 women; mean age [±SD] was 52.83±17.88 years). Pulmonary arterial pressure and severity of right ventricular dysfunction were assessed using RHC and echocardiography, respectively. RESULTS: Serum uric acid was higher in PH patients with severe RV dysfunction, compared to mild and moderate dysfunction (7.8mg/dl [IQR: 5.8-9.2] in severe dysfunction, versus 4.7 mg/dl [3.87-5.82] in mild dysfunction and 5 mg/dl [3.5-6.95] in moderate dysfunction. Serum uric acid was significantly correlated with pulmonary artery systolic pressure (r=0.51, P<0.001). Serum uric acid level also had a significant positive correlation with the World Health Organization functional class of the patients (r=0.49, P<0.001). Serum uric acid level greater than 5.7 mg/dl was found to be the most sensitive and specific points for predicting severe RV dysfunction in PH patients (sensitivity 76.6%, specificity 71.4%; AUC=0.79, P<0.001) . CONCLUSION: Serum uric acid is correlated with the severity of symptoms and RV dysfunction in patients with pulmonary hypertension. Further studies are recommended with larger sample size in this regard.

Treatment of Recurrent Prosthetic Mitral Valve Thrombosis with Reteplase: A Report of Four Cases.

Thrombosis is a life threatening complications of prosthetic mitral valves and is associated with high morbidity and mortality. Even in presence of systemic anticoagulation prosthetic valve thrombosis has an incidence of 0.5% to 8%. Recurrent prosthetic valve thrombosis and the resulting thrombotic occlusion require re-establishment of blood flow across the valve. While surgical repair is considered the classic first line treatment option for prosthetic valve thrombosis, intravenous thrombolysis has emerged as an acceptable alternative for the first episode of prosthetic valve thrombosis. Due to the limitation of using streptokinase in recurrent thrombotic events, fibrin-specific tissue plasminogen activators have been successfully utilized to treat cases of recurrent prosthetic valve thrombosis. In this case-series, we have reported four cases of recurrent prosthetic valve thrombosis that were successfully treated with Reteplase at our hospital.

Smoking and wound complications after coronary artery bypass grafting.

BACKGROUND: The harmful effects of smoking on the postsurgical wound healing disturbances have been widely investigated across various surgical procedures. These effects after coronary artery bypass graft (CABG) surgery have been less explored. We aimed to investigate the association of smoking and the wound healing problems in post-CABG patients. MATERIALS AND METHODS: We compared the incidence of wound complications in 405 smokers and 405 nonsmokers who underwent an elective CABG surgery. The incidence of leg and sternal wound complications was evaluated during the first 7 d as well as at a 6-wk postoperative visit. RESULTS: One hundred fifty-six leg wound complications were noted in 132 patients (16.3%). The overall rate of leg wound healing disturbances was significantly higher in smokers than those in nonsmokers (odds ratio, 1.47; 95% confidence interval, 1.109-4.019; P = 0.010). The incidence rates of leg wound edge necrosis and dehiscence were significantly higher in smokers compared with those in nonsmokers (3.7% versus 0.7%, P = 0.004 and 6.6% versus 0.7%, P < 0.0001, respectively). We found no significant differences between the incidence of postoperative leg wound infection, hematoma, wound edema, and seroma in active smokers and those who never smoked. Thirty-seven postsurgical sternal wound complications (4.6%) were developed in 33 patients (4.1%). The overall rate of sternal wound healing disturbances was similar between smokers and nonsmokers. There was a trend between the sternal wound dehiscence and smoking (P = 0.03); however, the other sternal wound complications were not associated with smoking. CONCLUSIONS: Smoking may contribute to the disturbances of wound healing, especially wound dehiscence, in post-CABG patients.

Hemodynamic and symptomatic improvement after delayed thrombolysis with Reteplase in a patient with massive bilateral pulmonary emboli.

Most patients surviving the acute phase of pulmonary embolism will recover with no residue. But, 2-4% of patients will progress to chronic thromboembolic pulmonary hypertension. In this group, usually a 'Honey moon' period is seen but a few may show progression with ongoing symptoms despite medical treatment. In this case report, we review a patient in whom delayed thrombolytic therapy was administered due to progressive symptoms after 21 days. Her condition was stabilized. The early posttreatment computed tomographic pulmonary angiography (CTPA) showed incomplete resolution, but after 6 months she was functional class I with a normal CTPA and echocardiography.

Application of intra-aortic balloon pump in resection and anastomosis of trachea.

The intra-aortic balloon pump (IABP) is a mechanical device used to assist cardiac circulatory function in patients suffering from cardiogenic shock, congestive heart failure, refractory angina and complications of myocardial infarction. While using IABP in cardiac surgery is well established, there are few studies on the utility of IABP support in high-risk cardiac patients undergoing non-cardiac surgery. Major non-cardiac surgeries are associated with high rates of cardiac complications in patients with advanced coronary disease. Recent case studies have reported favorable outcomes with the use of IABP support in non-cardiac surgery in patients with severe cardiac compromise. Using IABP may reduce cardiac complications by providing hemodynamic stability. Here, we present five cases of IABP use in high-risk cardiac patients undergoing resection and anastomosis of the trachea. IABP was inserted prior to induction of anesthesia in four of the cases, while IABP insertion was withheld in one case. In the four cases where IABP support was utilized, the IABP was removed between 6-48 hours postoperatively with no complications. The patient who did not undergo IABP insertion died on the 8th postoperative day due to uncontrollable pulmonary edema and progressive myocardial infarction. We also review the literature and discuss the role of IABP use in non-cardiac surgery.

Effect of pulmonary hypertension on outcome of pulmonary tuberculosis

Background: This study performed at the National Research Institute of Tuberculosis and Lung Disease, Tehran, Iran, aimed to evaluate the effect of concomitant pulmonary hypertension on the outcome of pulmonary tuberculosis. Methods: New cases of pulmonary tuberculosis were recruited for the study. Pulmonary hypertension was defined as systolic pulmonary arterial pressure ≥35 mm Hg estimated by transthoracic Doppler echocardiography. We assessed the relationship between pulmonary hypertension and mortality during the six-month treatment of tuberculosis. Results: Of 777 new cases of pulmonary tuberculosis, 74 (9.5%) had systolic pulmonary arterial pressure ≥35 mm Hg. Ten of them (13.5%) died during treatment compared to 5% of cases with pulmonary arterial pressure less than 35 mm Hg (p = 0.007). Logistic regression analysis showed that pulmonary hypertension and drug abuse remained independently associated with mortality (OR = 3.1; 95% CI: 1.44–6.75 and OR = 4.4; 95% CI: 2.35–8.17, respectively). Conclusion: A significant association was found between mortality and presence of pulmonary hypertension and drug abuse among new cases of pulmonary tuberculosis. .

Late relapse of multiple myeloma presenting as a right atrial mass.

Intracardiac involvement is a rare presentation of relapsed multiple myeloma. We present a case of lobulated mass in the wall of the right atrium, with extension to the inferior vena cava and obliteration and encasement of the interatrial septum in a 57-year-old man, a known case of multiple myeloma, with progressive exertional dyspnea and anasarca.

Effect of pulmonary hypertension on outcome of pulmonary tuberculosis.

BACKGROUND: This study performed at the National Research Institute of Tuberculosis and Lung Disease, Tehran, Iran, aimed to evaluate the effect of concomitant pulmonary hypertension on the outcome of pulmonary tuberculosis. METHODS: New cases of pulmonary tuberculosis were recruited for the study. Pulmonary hypertension was defined as systolic pulmonary arterial pressure ≥ 35 mm Hg estimated by transthoracic Doppler echocardiography. We assessed the relationship between pulmonary hypertension and mortality during the six-month treatment of tuberculosis. RESULTS: Of 777 new cases of pulmonary tuberculosis, 74 (9.5%) had systolic pulmonary arterial pressure ≥ 35 mm Hg. Ten of them (13.5%) died during treatment compared to 5% of cases with pulmonary arterial pressure less than 35 mm Hg (p=0.007). Logistic regression analysis showed that pulmonary hypertension and drug abuse remained independently associated with mortality (OR=3.1; 95% CI: 1.44-6.75 and OR=4.4; 95% CI: 2.35-8.17, respectively). CONCLUSION: A significant association was found between mortality and presence of pulmonary hypertension and drug abuse among new cases of pulmonary tuberculosis.

The Effect of Amlodipine and Sildenafil on the NT-ProBNP Level of Patients with COPD-Induced Pulmonary Hypertension.

Pulmonary hypertension (PH) is an important cause of heart failure in chronic obstructive pulmonary disease (COPD). The pro brain natriuretic peptide N-terminal (NT-proBNP) has been suggested as a noninvasive marker to evaluate ventricular function. However, there is no evidence to support the use of NT-proBNP in monitoring the benefits of vasodilators in COPD induced PH. Thus, we used NT-proBNP as a biomarker to evaluate the effect of oral vasodilators on cardiac function in COPD-induced PH. Forty clinically-stable PH patients were enrolled with history of COPD, normal left ventricular ejection-fraction (LVEF), right ventricular systolic pressure (RVSP) > 45 mmHg and baseline blood NT-proBNP levels >100 pg/mL. Patients were randomized into two groups, one group received sildenafil and second group were given amlodipine for two weeks. NT-proBNP and systolic pulmonary arterial pressure (systolic PA-pressure) were measured at the beginning and the end of study. Mean NT-proBNP level in the first group was 1297 ± 912 pg/mL before therapy and 554 ± 5 pg/mL after two weeks drug therapy, respectively. Similarly, in second group NT-proBNP level was 1657 ± 989 pg/mL and 646 ± 5 pg/mL before and after treatment. Amlodipine or sildenafil significantly reduced NT-proBNP levels in COPD-induced PH patients (p < 0.05). Our study shows that amlodipine and sildenafil have a similar effect on NT-proBNP levels. In both groups NT- proBNP levels were significantly reduced after treatment. Therefore, our findings support the potential benefits of treatment with vasodilators in COPD induced PH. Pulmonary hypertension, Chronic obstructive pulmonary disease, NT-proBNP, Amlodipine, Sildenafil.