Arshid Mir

Myocardial Infarction Associated With Use of the Synthetic Cannabinoid K2

Designer drugs have been problematic over the years. Products such as K2 and Spice, which contain synthetic cannabinoids, are marketed as incense and are widely available on the Internet and at various specialty shops. The effects are reported as cannabis-like after smoking them. In addition, use of these synthetic cannabinoids will not appear on a routine urine toxicology screen. Recently, K2 became a popular alternative to marijuana among youths. Health implications of these designer drugs are not completely understood. Little has been reported about the harmful effects of K2. We report here the first (to our knowledge) cases of myocardial infarction (MI) after smoking K2. Three patients presented separately to the emergency department complaining of chest pain within days after the use of K2. Acute MI was diagnosed in each case on the basis of electrocardiogram changes and elevated troponin levels. Coronary angiography was performed, and the results were normal for the first 2 patients. The incidence of ST-elevation MI is low among teenagers, and association with drug use should be suspected. Public education and awareness need to be heightened about the possible health implications of K2.

Hypertrophic Cardiomyopathy in a Neonate Associated with Nemaline Myopathy

Nemaline myopathy is a congenital nonprogressive skeletal muscle disorder with a characteristic rod body formation in the skeletal muscle fibers. Cardiac involvement in nemaline myopathy is rare, although both dilated and hypertrophic cardiomyopathy have been reported. We describe an infant diagnosed with hypertrophic cardiomyopathy and hypotonia on the first day of life. Muscle biopsy confirmed nemaline myopathy at 3 weeks of age. The diagnosis of nemaline myopathy precluded consideration of heart transplantation, thus shifting the focus to comfort care. This is the earliest presentation of hypertrophic cardiomyopathy reported in the literature in the setting of nemaline myopathy. The approach to determining an etiology for hypertrophic cardiomyopathy in an infant is reviewed.

Elevated troponin levels in previously healthy children: value of diagnostic modalities and the importance of a drug screen

BACKGROUND Myocardial injury in previously healthy children is rare, with a wide range of aetiologies. It is increasingly being identified on the basis of elevated troponin levels during routine evaluation of cardiorespiratory symptoms. Establishing the aetiology remains challenging because of the lack of an accepted work-up algorithm. Our objective was to delineate the contribution of diagnostic modalities and troponin patterns towards the final diagnosis. METHODS A retrospective chart review of previously healthy patients admitted to the Pediatric Cardiology Service with myocardial injury was carried out. Data analysed included echocardiograms, electrocardiograms, cardiac catheterisations, magnetic resonance imaging, drug screen tests, troponin values, and final diagnosis. RESULTS A total of 32 patients were identified. The diagnoses were: myocarditis in 16 patients, vasospasm due to drug use in seven, myopericarditis in six, anomalous coronary artery origins in two, and Prinzmetals angina in one patient. The electrocardiograms were abnormal in 27 of the 32 patients (84%), echocardiograms in 18 of the 32 patients (56%), cardiac magnetic resonance imaging in two of the four patients (50%), urine drug screen in five of the 25 patients (20%), and cardiac catheterisations in two of the 15 patients (13%). CONCLUSIONS Myocarditis is the most common aetiology of myocardial injury in children. Clinical history remains the basic screening tool; drug screens help identify coronary vasospasms secondary to drug use (22% of our cohort). Patients with anomalous coronaries had exertional symptoms. Initial troponin levels and progression were not diagnostic or prognostic. Catheterisation is of limited value and did not change management. Magnetic resonance imaging with gadolinium enhancement is probably the most useful test when initial evaluation is not diagnostic.

Intramural Coronary Artery in Truncus Arteriosus: Importance of Preoperative Echocardiographic Diagnosis and Impact on Surgical Planning

Coronary artery anomalies are known to be associated with truncus arteriosus (common arterial trunk). Delineation of coronary anatomy preoperatively is important to avoid complications intraoperatively and postoperatively. We report the case of a 12-year-old boy with repaired truncus arteriosus who presented with moderate to severe truncal valve regurgitation and severe conduit stenosis. He was noted to have intramural left main coronary artery on a transesophageal echocardiogram performed preoperatively.

Partial anomalous pulmonary venous return in a patient with turner syndrome

A 2-year old with a history of Turner Syndrome (XO), hypoplastic kidneys, hydronephrosis and gonadal dysgenesis had an abnormal screening echocardiogram which showed the following: bicuspid aortic valve, bilateral superior vena cava (SVC), dilated right atrium and ventricle, intact atrial septum and partial anomalous pulmonary venous return (PAPVR). By echocardiogram, the lower right and left pulmonary veins drained normally to the left atrium, the right upper pulmonary vein to the right SVC and the left upper pulmonary vein could not be identified. By cardiac magnetic resonance imaging (MRI), the findings agreed with echocardiogram with the exception that both the right upper and middle veins connected and drained anomalously to the right SVC (above the right pulmonary artery) and the unique finding of a left upper and lingular pulmonary veins draining directly to the left SVC to the coronary sinus. Of note a tiny bridging vein was present and a left azygous vein was not identified on MRI. In addition, an aortopulmonary collateral from the abdominal aorta to the right lung was identified by cardiac MRI. By cardiac catheterization, she underwent successful embolization of her aortopulmonary collateral and her pulmonary venous anatomy was confirmed. She underwent successful surgical repair of her defect and all findings were confirmed at the time of surgery. The PAPVR involving both the right and left sided pulmonary veins have been described, but the left sided veins usually drain into the innominate vein directly or via a vertical vein [1, 2]. This is the only report, to our knowledge, that has direct connection of the pulmonary veins to the left SVC draining inferiorly to the coronary sinus (Figs. 1, 2). Although bicuspid aortic valve and coarctation of the aorta are common cardiac lesions in patients with Turner Syndrome, there are multiple reports of anomalous pulmonary venous return with intact atrial septum [3, 4]. An adult study using cardiac MRI in Turner syndrome showed

Congenital ACTH Deficiency as a Cause of Hypoglycemia in a Newborn Infant

Isolated ACTH deficiency is a rare disorder, characterized by secondary adrenocortical insufficiency with low or absent cortisol production, normal secretion of pituitary hormones other than ACTH, and the absence of structural pituitary defects. Isolated ACTH deficiency has diverse clinical presentation and occasionally, if unrecognized, a fatal course. Unlike primary, secondary adrenal insufficiency is not associated with lack of aldosterone; thus, symptoms of mineralocorticoid deficiency are absent. Neonatal and childhood isolated ACTH deficiency may have genetic causes. A defect in the ACTH precursor, proopiomelanocortin (POMC), or in its cleavage enzyme, prohormone convertase, leads to defects in POMC-derived peptides (ACTH, MSH). Hypoglycemia, seizures, prolonged jaundice, and growth retardation are the prevalent symptoms in neonatal ACTH deficiency and failure to establish a timely diagnosis may be fatal. Identification of TPIT mutations allows prenatal diagnosis for families at risk and early glucorticoid therapy in order to prevent neonatal death. We describe a newborn who presented with severe hypoglycemia and was found to have low cortisol and ACTH levels. She was treated with supportive glucorticoid therapy, and the diagnosis was confirmed with an ACTH challenge test.

A rare cause of cardiomyopathy in an infant: middle aortic syndrome.

Coarctation of the abdominal aorta is a rare condition with a handful of cases reported in infancy. We report the case of a 4-month-old infant with severe abdominal coarctation who presented with dilated cardiomyopathy. Following successful transcatheter-based stenting of her abdominal aorta, her cardiomyopathy resolved.

Hypoplastic left heart syndrome with coronary-cameral fistulas: Echocardiographic demonstration of coronary artery steal and successful interventional treatment

We report a case of a patient with hypoplastic left heart syndrome with significant coronary-cameral fistulas and exertional symptoms from coronary steal. Symptoms resolved following successful coil occlusion of his left ventricle.

Hypoplastic left heart syndrome secondary to intrauterine rhabdomyoma necessitating single ventricle palliation

Rhabdomyoma, a benign hamartomatous tumor of the cardiac embryonic myocyte, is the most common intrauterine cardiac tumor and accounts for 0.12% of prenatal fetal studies. Fetal cardiac rhabdomyomas increase in size during second and early third trimester and spontaneously regress postnatally. The clinical presentation is usually benign, however, compromise of the ventricular outflow tract leading to decreased cardiac output and fetal death have been reported. We present a case of large cardiac rhabdomyoma in a fetus that might have caused complete left ventricular outflow tract obstruction and development of hypoplastic left heart syndrome (HLHS) necessitating postnatal single ventricle palliation therapy. The clinical course and outcomes of prenatally diagnosed cardiac rhabdomyoma are reviewed and theories of the development of hypoplastic left heart syndrome are explored.