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

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 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. Keywords: Congenital heart disease, Left main atresia, Coronary abnormalit

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

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

Ventricular Arrhythmia and Left Ventricular Dysfunction: A Rare Manifestation of Adrenal Adenoma

Primary aldosteronism is characterized by hypertension, suppressed plasma renin activity, increased aldosterone excretion, and hypokalemia with metabolic alkalosis. Ventricular arrhythmia is an uncommon finding. We report the case of a 46-year-old female who was referred to our center due to uncontrolled ventricular arrhythmia. The past medical history was positive for hypertension. On admission, echocardiography showed severe left ventricular (LV) dysfunction. Blood examination revealed severe hypokalemia. She had been diagnosed with acute coronary syndrome and decompensated heart failure elsewhere and was given diuretics. A diagnosis of primary aldosteronism due to adrenal adenoma was made according to laboratory findings and imaging modalities. The prompt management of bradycardia and correction of hypokalemia, along with surgical resection of adrenal adenoma, resulted in control of arrhythmias and improvement in LV function

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

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

Abstract 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