Mediastinal gray zone lymphoma in a pregnant woman presenting with cardiac tamponade.

BACKGROUND: Mediastinal gray zone lymphoma is a newly recognized rare B cell neoplasm, which is challenging in diagnosis and treatment. CASE PRESENTATION: In the current study, we aimed to report a 25-year-old pregnant woman at 25 weeks of gestation who presented with chronic cough and progressive shortness of breath, hypotension, tachycardia, and tachypnea. A large circumferential pericardial effusion with compressive effect on the right atrium and right ventricle and a large extracardiac mass with external pressure to mediastinal structures were seen on trans thoracic echocardiography. The emergency pericardiocentesis was performed with the diagnosis of cardiac tamponade. Also, CMR revealed a huge heterogeneous anterior mediastinal mass, and the pathology and the immunohistochemistry of the mass biopsy revealed gray zone lymphoma with positive CD3, CD20, CD30, CD45, PAX5, and negative CD15 expression. Three courses of chemotherapy with the CHOP regimen were performed with an acceptable response every three weeks before delivery. A caesarian section was performed at 37 weeks without any problem for the patient and fetus, and chemotherapy will be started three weeks after delivery. CONCLUSION: Cardiac tamponade as an emergency condition occurred in this pregnant patient by malignant pericardial effusion and mediastinal mass pressure. Accurate diagnosis and on time interventions caused a significant improvement and a successful delivery.

Post-Pulmonary Embolism Impairment Six Months after Acute Pulmonary Embolism: A Prospective Registry.

BACKGROUND: Little evidence is available on post-pulmonary embolism impairment (PPEI), a recently defined complication of pulmonary embolism (PE) encompassing dysfunctional clinical and imaging parameters. In the present study, we sought to evaluate its frequency with a focus on the main components. METHODS: In this prospective registry, we included patients with a confirmed diagnosis of acute PE and focused on those with initial right ventricular (RV) dysfunction. Their baseline, pre-discharge, and 6 month follow-up clinical and imaging characteristics were recorded. The main study outcomes were incomplete RV functional recovery, exercise capacity limitations (based on the 6 minute walk test), and their combination, which defines PPEI, within six months of acute PE. RESULTS: Of 170 consecutive patients with a confirmed diagnosis of acute PE, 123 accepted to participate in the follow-up study, of whom 87 had initial RV dysfunction. The 6 month rates of incomplete RV functional recovery, signs of an intermediate-to-high echocardiographic probability of PH, and exercise limitations were observed in 58.6, 32.1, and 45.9%, respectively. A total of 22 (25.2%; 95% CI 15.5-34.4%) patients had PPEI. The RV/LV ratio and the fractional area change on discharge after acute PE were more often impaired among patients with incomplete RV recovery, exercise limitations, and a high probability of PH at 6 months. In contrast, an initial impaired RV diastolic function indices appeared to characterize patients with a limited exercise capacity at 6 months. DISCUSSION: PPEI affects one fourth of patients surviving acute PE with half of them presenting with RV dysfunction or exercise limitations.

Balloon-Expandable Cheatham-Platinum Stents Versus Self-Expandable Nitinol Stents in Coarctation of Aorta: A Randomized Controlled Trial.

OBJECTIVES: This study sought to compare the safety and efficacy of the balloon-expandable stent (BES) and the self-expandable stent (SES) in the endovascular treatment of coarctation of aorta. BACKGROUND: Coarctoplasty with stents has conferred promising results. Although several nonrandomized studies have approved the safety and efficacy of the BES and the SES, no high-quality evidence exists for this comparison. METHODS: In the present open-label, parallel-group, blinded endpoint randomized pilot clinical trial, adult patients with de novo native aortic coarctation were randomized into Cheatham-platinum BES and uncovered nitinol SES groups. The primary outcome of the study was a composite of procedural and vascular complications. The secondary outcomes of the study consisted of the incidence of aortic recoarctation, thoracic aortic aneurysm/pseudoaneurysm formation, and residual hypertension at a 12-month follow-up. RESULTS: Among 105 patients who were screened between January 2017 and December 2019, 92 eligible patients (32 women [34.8%]) with a median age of 30 years (IQR: 20-36 years) were randomized equally into the BES and SES groups. The composite of procedural and vascular complications occurred in 10.9% of the BES group and 2.2% of the SES group (odds ratio: 0.18; 95% CI: 0.02-1.62; P = 0.20). Aortic recoarctation occurred in 5 patients (5.4%), 3 patients (6.5%) in the BES group and 2 patients (4.3%) in the SES group (odds ratio: 0.65; 95% CI: 0.10-4.09; P = 0.64). Only 1 patient (1.1%) was complicated by aortic pseudoaneurysm. Hypertension control was achieved in 50% of the study population, with an equal distribution in the 2 study groups at the 12-month follow-up. CONCLUSIONS: Both the BES and the SES were safe and effective in the treatment of native coarctation.

Incidental detection of ruptured sinus of Valsalva aneurysm and coronary abnormality in a patient with penetrating chest trauma.

Aortic sinus of Valsalva aneurysm is a rare congenital or acquired anomaly with a potential for rupture into adjacent cardiac chambers. Ruptured aneurysm of the sinus of Valsalva leads to volume overload of the receiving chamber and should be repaired as soon as the diagnosis is made. Here, we present a young patient with history of chest trauma leading to discovery of an asymptomatic ruptured sinus of Valsalva aneurysm.

Spontaneous Intramural Esophageal Hematoma Secondary to Thrombolysis in the Setting of Pulmonary Embolism.

Intramural hematoma of the esophagus (IHE) represents a rare condition on the spectrum of esophageal injuries. The most common symptoms are hematemesis, epigastric pain or retrosternal chest pains, odynophagia, and dysphagia. Early recognition of IHE is important as it may mimic other diseases such as myocardial infarction, pulmonary embolism, Mallory-Weiss tears, Boerhaave's syndrome, ruptured aortic aneurysms, and aortic dissection. Computed tomography is the preferred investigation method, and treatment is usually conservative. We herein present 2 cases of IHE associated with catheter-directed thrombolysis in the setting of pulmonary embolism.

Central Nervous System and Cardiac Involvement in the Hypereosinophilic Syndrome: A Case Report.

Hypereosinophilic syndrome is a rare entity and heterogeneous group of disorders characterized by hypereosinophilia and organ involvement. In this study, we presented a 49-year-old woman with cardiac tamponade in the context of Hypereosinophilic syndrome. Identifying hypereosinophilia as the underlying cause can have tremendous clinical implications for rapid initiation of appropriate treatment to minimize further end organ damage.

Pulmonary artery-focused contrast echocardiography with supplemental oxygen(PCESO) for echocardiographic diagnosis of anomalous origin of left coronary artery from pulmonary artery: Novel use of an old technique.

Pulmonary artery-focused agitated saline contrast echocardiography unveils tricky cases of ALCAPA by the entry of microbubbles into the left coronary artery (LCA) during systole and retrograde flow from LCA into the main pulmonary artery during diastole. Associated pulmonary hypertension, if present, augments the former flow and supplemental oxygen increases the latter.

Isolation of the left brachiocephalic artery revisited: A 52-year literature review and introduction of a novel anatomic-clinical-prognostic classification.

Isolation of the left brachiocephalic artery (ILBA) is an extremely rare anomaly of aortic arch with diverse manifestations in the neurologic system, heart, and left upper arm. This anomaly is defined as the absence of connection of the left brachiocephalic artery (LBA) to aortic arch and connection of LBA to pulmonary artery (PA) through a patent arterial duct (PAD). However, this definition is not inclusive of all cases. Not only are there inconsistencies in the definition and terminology of this aortic arch anomaly but also there is no classification for this anomaly despite its heterogeneous nature in terms of anatomy, clinical presentation and prognosis. We performed a 52-year comprehensive literature review in the period between 1966 and 2018. Our inclusion criteria were any manuscript that included a case report or case series, with confirmed diagnosis of ILBA. All quantitative data were analyzed using descriptive analysis by SPSS version 21 (IBM SPSS Statistics, USA). Results were presented as mean ± standard deviation and median. Based on the presence or absence of connection of LBA to PA and the number of sources of steal from the LBA, we classified ILBA into three types: single-steal type with no connection of LBA to PA and single source of blood flow steal from LBA through the left subclavian artery (LSCA), double-steal type with connection of LBA to PA through PAD and two sources of steal through LSCA and arterial duct (AD), and triple-steal type with bilateral PADs and therefore, three sources of blood flow steal from LBA including the LSCA and the double ADs. Patients with single-steal type have the best prognosis and present latest with symptoms of cerebrovascular insufficiency or left arm claudication. The oldest reported patient was 69 years of age with symptoms of dizziness and near syncope. No death was reported in these patients. Double-steal type is the most common type and is often associated with genetic syndromes and/or extracardiac anomalies. Triple-steal type is the rarest type with the earliest presentation and worst prognosis. The oldest reported patient was 60 days of age. All reported cases had cardiac symptoms, pulmonary overcirculation, pulmonary hypertension, and fatal outcome.

Congenital atresia of left main coronary artery.

Congenital atresia of the left main coronary artery is a very rare form of coronary anomalies with poor clinical outcomes if left untreated. Patients require surgical correction by coronary bypass grafting after diagnosis. Here we report a case of congenital left main atresia in a 36 years old woman who had previous heart surgery with this anomaly having gone undetected.

Asymptomatic young man with an incidental murmur.

CLINICAL INTRODUCTION: A 32-year old man was referred to our institution for transthoracic echocardiography (TTE) following detection of an incidental murmur on physical examination before blood donation. He was asymptomatic with no significant medical history. Physical examination revealed dual heart sounds with a grade II/VI systolic murmur heard in the left sternal border. An ECG was in normal sinus rhythm. TTE was performed (figure 1A-C, online supplementary videos 1-4) followed by cardiac CT angiography (CTA) (figure 1D,E).heartjnl;104/15/1307/F1F1F1Figure 1(A) Transthoracic echocardiography, parasternal left ventricular long axis view. (B) Colour Doppler of modified short axis in the mid-left ventricular level. (C) Doppler flow velocity profile. (D) Cardiac CT angiography (CTA) sagittal reconstruction. (E) Three-dimensional CTA reconstruction of the heart. QUESTION: What is the diagnosis?Pericardial cyst.Ventricular septal defect.Kawasaki.Anomalous left coronary artery from pulmonary artery (ALCAPA).

Pitfalls of stenting coarctation of an angulated right circumflex aortic arch in Goldenhar syndrome.

We report stenting of coarctation of an angulated right circumflex aortic arch (RCAA) using four Cheatham Platinum stents in a child with Goldenhar syndrome. Difficulties in measuring the accurate length of the curved and narrowed transverse aortic arch marked discrepancy between the luminal diameters of the long narrow transverse arch and wide descending thoracic aorta, increased displacement force caused by the 90° bend between the two parts resulted in repeated stent migrations. We discuss the tips to avoid distal stent migration in the setting of an angulated RCAA.

Transcatheter coil embolization of iatrogenic coronary artery-left ventricle fistula after mitral valve replacement.

Acquired causes of coronary fistulas are rare disorders and may develop following coronary atherosclerosis, infection, or trauma (iatrogenic). Iatrogenic coronary fistulas may be acquired secondary to surgical or nonsurgical interventions. We describe a case of an iatrogenic coronary artery-left ventricle fistula following mitral valve replacement surgery, presented with ventricular arrhythmia and heart failure. In a unique technique, we implanted three coils with the aid of a Scepter C balloon with inflation at the ostial portion of the left circumflex artery.

Congenital Aortico-Left Ventricular Tunnel: A Case Report of a Rare Cause of Aortic Regurgitation in Adults.

The aortico-left ventricular tunnel is a rare congenital abnormality resulting in a pathologic connection between the aorta and the left ventricle. It often presents during infancy or early childhood as a cardiac failure symptom or an incidental finding of a cardiac murmur due to severe aortic regurgitation. It is, however, also occasionally found in asymptomatic adults. We describe a 20-year-old female presenting with palpitations in whom clinical evaluations with echocardiography and computed tomography angiography led to the diagnosis of severe aortic regurgitation caused by a tunnel connecting the right sinus of the aorta to the left ventricle. The patient underwent successful obstruction of the tunnel with an autologous pericardial patch and the repair of the dilated aortic root via the reduction aortoplasty technique. She was discharged on the 5th postoperative day with no complications. At 1 month's follow-up, she remained asymptomatic and echocardiography showed aortic valve competence with no residual regurgitation.

Percutaneous treatment for aneurysmal coarctation of the aorta with covered stenting: a case report.

The coarctation of the aorta (CoA) is rare in adulthood. Diagnosis is made by clinical suspicion and physical findings such as blood pressure difference between the upper and lower extremities, pulse delay in the femoral artery, and systolic murmur over the thoracic spine. The CoA in adulthood and in patients with associated aneurysm is challenging and different complications even with proper treatment can occur. Covered stents are indicated in concomitant aneurysm, older age, and tight coarctation. A 26-year-old male with resistant hypertension due to a CoA diagnosed by computed tomography angiography referred to our center for an attempted stent implantation. Cardiac catheterization and aortography revealed a long CoA after the origin of the left subclavian artery with a 60 mmHg gradient. Moreover, there was a large aneurysm in the site of the coarctation. Under general anesthesia and fluoroscopic guidance, two balloon-expandable covered Cheatham-Platinum stents (size 18 in 44 millimeters and size 18 in 50 millimeters) were successfully implanted across the CoA with no residual gradient. On 2 years' follow-up, the patient had no symptoms except for mild hypertension. In this patient, the use of a covered stent within the aneurysm was safe and effective.

Aortic runoff as a sign of intracranial arteriovenous malformation: report of two cases.

BACKGROUND: Intracranial arteriovenous malformation rarely causes pulmonary hypertension and congestive heart failure in the newborn. Its diagnosis is challenging because cardiomegaly may suggest an intra-cardiac structural lesion. CASE PRESENTATION: We present two newborns, one 2-day-old male and the other 11-day-old female, with intracranial arteriovenous malformation and misdiagnosis of congenital heart disease. CONCLUSION: Precise echocardiography revealed the secondary signs of cranial arteriovenous malformation and had the major role in early diagnosis.

From an isolated right ventricular thrombus to the diagnosis of the hypereosinophilic syndrome.

This case concerns a 58-year-old male who presented with abdominal pain, anorexia, nausea, and exertional dyspnea of one month's duration. On further evaluation peripheral eosinophilia was conspicuous and eosinophilic infiltration was found in the gastrointestinal system and bone marrow. Echocardiography showed an isolated right ventricular thrombus with tricuspid valve involvement. Cardiac magnetic resonance imaging helped to confirm the diagnosis of cardiac involvement in hypereosinophilic syndrome without requiring a cardiac biopsy.