Sudden Cardiac Arrest as first Manifestation of Malignant Mitral Valve Prolapse in a Young Patient: A Case Report and Review of the Literature.

Mitral valve prolapse (MVP) is a primary valvular disease of the mitral valve with a prevalence of 2.4% of the general population. Valve abnormalities range from simple fibroelastic deficiency of the leaflets to diffuse myxomatous degenerative changes. MVP is a usually a benign condition. However, the scattered reports of sudden cardiac death in patients with MVP in the absence of severe mitral insufficiency or coronary artery disease suggest the existence of a malignant phenotype of MVP. We report a case of a young female who survived life-threatening arrhythmias and cardiac arrest and was found to have characteristic features of the malignant phenotype of MVP and had an implantable cardioverter defibrillator as a secondary prevention. LEARNING POINTS: Malignant MVP may be associated with life-threatening arrhythmias and sudden cardiac death.MVP is not always a benign condition, and physicians should be aware of the diagnostic criteria for malignant MVP.Echocardiography and cardiac magnetic resonance are crucial diagnostic methods to detect signs suggestive of malignant MVP.

Angina Pectoris as a Manifestation of ALCAPA Syndrome in a 20-Year-Old Female: A Case Report and Review of Literature.

Anomalous left coronary artery from the pulmonary artery (ALCAPA) is considered a rare congenital heart disease where the take-off of the left coronary artery abnormally originates from the pulmonary artery instead of left aortic sinus. It is associated with a high mortality rate in the first year of life and sudden death in adults if left untreated. We report an adult form of ALCAPA syndrome in a 20-year-old female who presented with anginal pain for the previous few months. Unfortunately, the patient was hesitant to have surgery at the time. LEARNING POINTS: The abnormal origin of the left coronary artery from the pulmonary artery (ALCAPA) is rare and potentially fatal coronary congenital disease, accounting for 0.5% of all congenital heart diseases; it is associated with poor outcomes if left untreated.ALCAPA is classified into infantile and adult forms. The prevalence of adult individuals with ALCAPA syndrome has significantly increased as a result of recent developments in non-invasive cardiac imaging.The prevalence of sudden mortality in childhood and the early stages of adulthood makes surgery the preferred treatment, and coronary reimplantation surgery is considered the surgical procedure of choice.

Cardiac Hydatid Disease with Palpitations as the Only Presenting Complaint: The Egg in the Nest.

Hydatid disease, also known as cystic echinococcosis, is a parasitic infection caused by the larval stage of Echinococcus granulosus. It usually invades organs such as the liver and the lungs but can affect any organ. Isolated cardiac involvement is a rare manifestation. We present a case of isolated left ventricle hydatid cyst with negative serological tests, which was treated with surgical removal and confirmed by histopathological examination. LEARNING POINTS: Isolated cardiac hydatid disease is rare and accounts for only 0.5-2% of cases among infected patients.Cardiac hydatidosis may present with a wide variety of clinical manifestations ranging from no symptoms to sudden death; palpitations may be the only complaint.Surgical excision is the treatment of choice for cardiac hydatid disease to avoid high-risk complications.

A PREGNANT WOMAN WITH AN EXTRA CARDIAC CAVITY: AN ACQUIRED IATROGENIC COMPLICATION - A CASE REPORT AND LITERATURE REVIEW.

According to the modified World Health Organization (WHO) classification, mechanical valves pose a high maternal risk and complications for pregnant women with heart disease. Left atrial appendage aneurysm (LAAA) is a rare condition that can manifest clinically in several ways or remain silent for a long time and can be either congenital or acquired. We present the case of a pregnant woman who had a LAAA discovered several years after her last mitral valve replacement. LEARNING POINTS: Left atrial appendage aneurysm is a rare entity and, in most cases, is congenital due to poor myocardial contractility of dysplastic pectinate muscles.Clinical manifestations range from an asymptomatic course with an incidental finding on echocardiography up to serious sequelae such as cardioembolic manifestations.The treatment approach includes a conservative strategy using anticoagulation and a surgical strategy with aneurysmectomy.

Antioxidant activity in off and on-pump coronary artery bypass grafting and valve replacement surgery.

OBJECTIVES: Cardiac surgeries initiate oxidative stress, increasing organ dysfunction development and mortality. The present study investigated the superoxide dismutase (SOD) activity, as an antioxidant enzyme, in patients undergoing coronary artery bypass (CABG) and heart valve replacement surgeries. METHODS: A prospective study was performed on patients with proven coronary artery disease (CAD) or valvular heart disease (VHD) candidates for on- or off-pump CABG and valve replacement surgery. Serum SOD activity was measured preoperatively, at 24 h postoperatively, and at the time of discharge. In addition, echocardiography was performed before surgery and at discharge. RESULTS: A total of 48, 51, and 47 patients were enrolled in the on-pump, off-pump CABG, and valve replacement groups, respectively. Baseline serum SOD activity showed no significant association with BMI, age, and blood pressure in either CAD or VHD patients. The SOD values decreased at the 24 h postoperative time and then increased at the time of discharge in all groups, except for a slight decrease in the on-pump group. The changes in serum SOD values were not significantly different for the three surgical groups. CONCLUSIONS: The serum SOD activities fell significantly after CABG and valve replacement surgery. Further investigation is emphasized for the role of SOD in oxidative stress after cardiac surgery.

The effects of low-dose anthracycline-based chemotherapy on the levels of serum NT-proBNP level and left ventricular systolic and diastolic dysfunctions: A prospective observational study.

Background and Aims: The present study aimed to evaluate the relationship between serum N-terminal (NT)-pro-brain natriuretic peptide (BNP) levels and incidence of left ventricular (LV) systolic and diastolic dysfunction in patients who underwent low-dose anthracycline chemotherapy. Methods: This observational, prospective study was conducted on all patients with proven breast cancer, Hodgkin lymphoma, or non-Hodgkin lymphoma and no history of previous cardiac or any chronic diseases who were candidates for low-dose anthracycline chemotherapy from March 2017 to February 2018. We evaluated the serum NT-proBNP level and performed trans-thoracic echocardiography at baseline, 6, and 9 months after the chemotherapy. Results: Among 57 patients who successfully finished the study, 13 (22.8%) patients were men and 44 (77.19%) patients were women, and the total mean age was 52 ± 14.86 years. Our results revealed that the LV systolic and diastolic function and NT-proBNP mean levels were in the normal range at all three measured times. Additionally, no significant differences were observed between the levels of NT-proBNP, LV systolic, and diastolic function at baseline, 6, and 9 months after chemotherapy with low-dose anthracycline (p = 0.6, 0.1, and 0.4, respectively). Conclusion: Following low-dose anthracycline chemotherapy, none of our patients encountered LV systolic and diastolic dysfunctions and changes in serum NT-proBNP level. However, further studies with a larger population, longer follow-up duration, and higher dosage of anthracyclines are required to determine the effects of low-dose anthracycline on NT-proBNP level and LV systolic and diastolic functions.

Changes in serum zinc and copper concentrations in patients with cardiovascular disease following cardiac surgery.

The trace elements copper (Cu) and zinc (Zn) are essential for maintaining oxidative balance, and cardiac surgery is known to provoke an increase in oxidative stress. We investigated the variations in serum Zn and Cu concentrations before and after surgery in patients undergoing on- and off-pump CABG and heart valve replacement. We performed a prospective study on patients undergoing on- or off-pump CABG, or heart valve replacement surgery (48, 51, and 47 patients, respectively). Venous blood samples were obtained, and serum Cu and Zn concentrations were measured preoperatively, 24 h postoperatively, and the time of discharge. In addition, echocardiography was carried out on all patients before surgery and again on the day of discharge. We found the temporal changes in Cu, Zn, and Zn/Cu ratio were significantly different in all three groups of surgery (p < 0.05). In each group, Cu and Zn values and Zn/Cu ratio decreased at the 24-h postoperative time and rose at the discharge time. There were no significant differences between surgery groups in the changes induced in Zn or Cu values (p > 0.05). In conclusion, the concentrations of Cu and Zn were markedly reduced after on- and off-pump CABG and valve replacement surgery. This may suggest that supplementary Zn and Cu administration could be beneficial during open-heart surgeries. However, more long-term studies with more patients are needed to confirm this hypothesis.

A comparative study on the results of beating and arrested heart isolated tricuspid valve surgery: A cross-sectional study.

Background and Aims: The isolated tricuspid valve surgery is performed using beating or arrested heart surgery. The present study aimed to evaluate and compare the results of patients who underwent isolated tricuspid valve surgery using the beating and arrested heart techniques. Methods: In this retrospective observational study, all patients undergoing isolated tricuspid valve surgery between 2011 and 2018 were included. The patient's demographic information, echocardiographic, surgery, and hospitalization results were collected, and the EuroSCORE was calculated from the patient's medical records. Results: Fifty-three patients were included in the study of which 21 (39.63%) and 32 (60.37%) underwent a beating heart and arrested heart surgeries, respectively. There were no significant differences between the two studied groups in the echocardiographic results before and after the surgery, total hospitalization days (p = 0.56), and the mortality in the first 30 days after the surgery (p = 0.152). However, the duration of surgery (p < 0.001), EuroSCORE (p = 0.005), and patients who underwent lateral thoracotomy (p = 0.007) were notably lower in the arrested heart than in the beating heart group. In contrast, the duration of cardiopulmonary bypass and hospitalization in an intensive care unit after the surgery was remarkably lower in the beating heart than in the arrested heart group (p < 0.001 for both cases). Conclusion: Although there were some significant differences between the two kinds of surgery techniques, it seems superficial that made a particular decision to consider each method's superiority. Therefore, further studies with larger populations and meta-analyses are required to recommend the preferred method for the surgeons certainly.

Three-dimensional echocardiography of a tricuspid valve papillary fibroelastoma.

Papillary fibroelastomas (PFE) on tricuspid valve (TV) are rare, accounting for fewer than 11% of all PFE. Most often they are asymptomatic, and detect on mitral valve. We report a case of a typical chest pain found to be due to papillary fibroelastoma of the tricuspid valve.

Surgical embolectomy in the management of massive and sub-massive pulmonary embolism: The results of 30 consecutive ill patients.

BACKGROUND: Despite the improvement in the diagnosis and treatment of acute pulmonary embolism, it is yet a common clinical problem. The actual role of open embolectomy has not been well understood. The present report aimed to extrapolate the outcome of early open pulmonary embolectomy in a number of patients with acute (sub) massive pulmonary embolism (AMPE/ASMPE). METHODS: A prospective study was performed on 30 patients who underwent emergency embolectomy at Ghaem Hospital, Mashhad, Iran during January 2005 to November 2012. All patients with an indication for pulmonary embolectomy according to recent American Heart Association guideline were enrolled in this study. Echocardiographic features, pulmonary artery pressure, and right ventricular (RV) diameter were recorded. The patients were followed up monthly by two cardiologists. RESULTS: Indications for operation in descending order consisted of contraindication for fibrinolytic therapy (30%), failure to respond to fibrinolysis (26.66%), cardiopulmonary arrest (20%), patent foramen ovale (20%), right atrium clot (10%), and cardiogenic shock (10%). Mean pulmonary artery pressures were 52.26 ± 6.54 and 29.43 ± 2.87 mmHg before and after the operation, respectively (P < 0.0001). RV function and diameter improved significantly after surgery (P < 0.0001 and < 0.0001, respectively). Complete follow-up was performed in all surviving patients. All patients survived the operation, except one who died 2 days after surgery due to profound hypotension. CONCLUSION: Short and long-term outcomes of early open embolectomy seemed to be satisfactory in high-risk patients presenting high clot burden in central pulmonary arteries. This study demonstrated that pulmonary embolectomy may play a promising role in the management of AMPE and ASMPE and recommended for future clinical trials.

Surgical embolectomy versus thrombolytic therapy in the management of acute massive pulmonary embolism: Short and long-term prognosis.

OBJECTIVE: Comparison between surgical embolectomy and thrombolytic therapy in patients suffering from acute massive pulmonary embolism (AMPE). BACKGROUND: Prompt treatment of AMPE is necessary, although optimal management is a matter of debate. METHODS: Patients with AMPE were assigned to either thrombolytic therapy or pulmonary surgical embolectomy. Early and late mortality, systolic pulmonary artery pressure (SPAP), right ventricular diameter (RVD) and bleeding complications were evaluated. RESULTS: Seventy eight patients were treated with thrombolytic therapy and 30 patients underwent surgery. The difference between pre-intervention and third-day post-intervention in terms of RVD and SPAP was significantly greater in patients under surgical embolectomy (P < 0.001). There was a significant decline in RVD and SPAP in both groups during follow-up (P < 0.001). Mortality rate in the surgical embolectomy group was lower than the thrombolytic group although not significantly. CONCLUSION: Early surgical treatment was associated with fewer complications in comparison to thrombolytic therapy.

An unusual biatrial cardiac myxoma in a young patient.

This is a report of a biatrial cardiac myxoma in a young man with a 10-month history of exertional dyspnea and palpitation. The echocardiogram revealed biatrial myxoma prolapsing through the mitral and tricuspid valves during diastole. All cardiac chambers were enlarged and dysfunctional. The electrocardiogram revealed a rapid ventricular response with atrial flutter rhythm. The masses were resected and diagnosed as myxoma by a histological examination. The follow-up echocardiogram revealed significant improvement in ventricular function and reduction in the cardiac chambers' volume. There was no evidence of myxoma recurrence. The most probable cause of the patient's heart failure was considered to be tachycardia-induced cardiomyopathy.

Congenital coronary artery anomaly simulating a ventricular septal defect.

Anomalous origin of the circumflex artery of the right sinus of Valsalva is a rare finding which may be present with other cardiac malformations. A 19-year-old man presented with syncope. A transthoracic echocardiogram revealed discrete subaortic stenosis with a small defect just below the aortic valve, suggesting a ventricular septal defect. Transesophageal echocardiography showed anomalous origin of the circumflex artery from the right sinus of Valsalva. This was confirmed by coronary angiography. The patient underwent successful web resection without concomitant coronary surgery. Failure to demonstrate a coronary artery anomaly can be misleading for surgeons and perilous for patients.

A case of left atrial myxoma with unusual tumor vascularity.

A 69-year-old woman presented with a 2-week history of chest pain and mitral and aortic valve replacement 15 and 5 years earlier. Transthoracic echocardiography revealed a well-seated monoleaflet mitral valve and a bileaflet aortic valve with normal function. Cardiac catheterization showed 3-vessel coronary artery disease and an abnormal cluster of new vessels derived from the left atrial circumflex and posterior ventricle branches. Two- and 3-dimensional transesophageal echocardiography identified a semi-mobile oval left atrial mass with multiple internal cystic spaces, attached to the fossa ovalis region in the interatrial septum. The mass was resected. The pathological diagnosis was atrial myxoma.

An improved technique for pulmonary endarterectomy.

We report a modified technique for pulmonary endarterectomy (PEA) on a 67-year-old man with chronic thromboembolic pulmonary hypertension (CTEPH) who presented with dyspnea. He was referred to our medical center for coronary artery bypass grafting. CTEPH had not been detected in his first visit to another medical center, but upon re-evaluation, the diagnosis was confirmed. PEA was performed with a modified method, which seems to be safe and suitable for the removal of clot and fibrotic materials. Iatrogenic dissection was performed with normal saline injection in the pulmonary artery, and then, the clot was removed completely. Although the technique may not be applicable for all cases, it can be used as an alternative to using an aspirating dissector and a pair of forceps.

Carcinoid heart disease and the utility of 3D trans-thoracic and trans-esophageal echocardiography: Two clinical cases.

Carcinoid tumors are rare neuroendocrine malignancies. We present two cases of metastatic carcinoid tumors, complicated by carcinoid syndrome and by cardiac valve involvement. Carcinoid syndrome is characterized by secretory diarrhea, episodic flushing, and bronchospasm. Cardiac involvement occurs in up to 50% of patients with metastatic carcinoid tumors which commonly causes abnormalities of the right sided valves. Echocardiography is the best available and non invasive technique for diagnosis. Characteristic features of carcinoid heart disease are thickened, shortened, retracted, and fixed or partially fixed valve leaflets. Three-dimensional (3D) echocardiography provided an en face view of pulmonary and tricuspid valve, not obtainable by two-dimensional echocardiography, and improved delineation of the relationship between these structures and cardiac chambers.

Frequency of ischemic mitral regurgitation after first-time acute myocardial infarction and its relation to infarct location and in-hospital mortality.

BACKGROUND: Ischemic mitral regurgitation (IMR) is a common complication after acute myocardial infarction (AMI). We aimed to investigate the frequency of IMR following first-time AMI and its association with infarct location, in-hospital mortality, and complications. METHODS: From September 2011 to November 2012, all patients with a diagnosis of first-time acute ST-elevation MI were enrolled in the study. Patients with previous MI and heart failure, organic mitral valve disorders, and previous mitral surgery were excluded from the study. The patients' baseline characteristic, echocardiographic parameters, and complications were recorded. The frequency of IMR after AMI and its relation to infarct location and in-hospital mortality were evaluated. RESULTS: Altogether, 250 patients (180 male) at a mean age of 60.21 ± 12.90 years were studied. IMR was detected in 114 (45%) patients. There was no association between the presence of MR and gender, systemic hypertension, smoking, diabetes mellitus, or body mass index; however, serum LDL-cholesterol and triglyceride levels were significantly higher in the patients with IMR . The most frequent territory of MI was anterior in the patients without MR, while the anterolateral territory was the most common one in the patients with IMR. The patients with IMR had more reduced left ventricular ejection fraction, more elevated left ventricular end-diastolic pressure, and higher pulmonary arterial pressure (p values < 0.001, < 0.001, and < 0.001, respectively). Stage III diastolic dysfunction was more frequent in the patients with IMR. All the deaths occurred in the IMR patients, who also had more complicated AMI. CONCLUSION: IMR following AMI is highly prevalent, and it complicates about half of the patients. Regarding its relation to the AMI complications, assessment of the MR severity is necessary to make an appropriate decision for treatment.