İlkay Erdoğan

Clinical experiences of patients with catecholaminergic polymorphic ventricular tachycardia.

Catecholaminergic polymorphic ventricular tachycardia is a rare entity that can occur in children without cardiac disease and with a normal QT interval. It may cause syncope, convulsions, and sudden death during physical activity or emotional distress. We report the clinical features, treatment, and follow-up of 16 children with this diagnosis, emphasizing the potentially fatal nature of the disease.The mean age of patients at the onset of symptoms and at the time of diagnosis was 7.8 plus or minus 2.5 years, and 10.6 plus or minus 3.5 years, respectively. Syncope was the main complaint in 11, and 7 were treated as erroneously as having epilepsy. Diagnosis was confirmed by exercise and/or infusion of isoproterenol. Once the diagnosis was made, we started propranolol in all patients, and added verapamil if ventricular tachycardia was still inducible on a treadmill exercise test. An intracardiac defibrillator was implanted in 4 patients. Of the 16 patients, 4 died suddenly, giving a rate of mortality of 25%. In 2 of those dying suddenly, there was evidence of poor compliance to the recommended treatment. Another 2 patients had been resuscitated because of sudden cardiac arrest.Catecholaminergic polymorphic ventricular tachycardia must be considered in the differential diagnosis of syncope in children without heart disease but with a normal QT interval. Medical treatment with propranolol and verapamil may decrease the incidence of arrhythmia. Implantation of intracardiac defibrillators should be considered in those resistant to drug therapy. Delay in diagnosis, and inadequate treatment, can result in sudden cardiac death.

Congenital Aortic Stenosis: A Novel Technique for Ventricular Pacing During Valvuloplasty

Objectives: The main objective of this study is to describe a new technique for rapid ventricular pacing to maintain balloon stability during balloon aortic valvuloplasty (BAV) without using transvenous temporary pacemaker electrode. Background: The safety and efficacy of rapid right ventricular pacing to facilitate balloon stability during BAV has been previously reported. However, it necessitates an additional femoral venous access, an additional sheath, and a temporary pacemaker electrode. Methods: This was a prospective pilot study. Rapid ventricular pacing was performed through back‐up guidewires inserted into the left ventricle for balloon advancement and by an adhesive patch placed on the back of the patient. The technique was performed during BAV procedure in all of 15 consecutive children diagnosed as congenital aortic stenosis. Pacing was performed at a rate decreasing systolic aortic pressure to the point of 40–50% of baseline. Results: The technique was successful in all patients. Effective capture, stable pacing, and balloon stability were achieved in all children using very low outputs. No sustained arrhythmias or other procedure‐related complications occurred. Mean aortic valve gradient decreased from 68.5 ± 20.4 mm Hg to 20.4 ± 10.2 mm Hg. Degree of aortic regurgitation progressed from grade 0 to 1 in three patients and remained unchanged in 12 patients. Conclusions: This study demonstrated that back‐up guidewires can be used effectively and safely for pacing during BAV procedures. This technique omits probable complications related to a second vascular access and may shorten the procedure time and decreases costs by eliminating the use of an additional sheath and a temporary pacemaker electrode.

Transposition of the great arteries and cor triatriatum: a rare combination.

In this case report, we present a 5-month-old girl diagnosed with a unique combination of transposition of the great arteries and cor triatriatum sinistra. A 1-day-old female patient presented to our hospital with cyanosis since the early neonatal period. We confirmed transposition of the great arteries by echocardiography. The patient underwent arterial switch operation on day 8 and was discharged on day 35. After 5 months of the operation, the patient had a lower respiratory tract infection and was unable to gain weight. Echocardiography revealed mild neopulmonary regurgitation, minimal neoaortic regurgitation, and pulmonary arterial hypertension. In addition, a fibrous membrane was also seen dividing the left atrium. The patient was diagnosed with cor triatriatum and underwent successful resection of the membrane.

A Report of Brugada Syndrome Presenting with Cardiac Arrest Triggered by Verapamil Intoxication

Background: Brugada syndrome is a disease characterized by a specific electrocardiographic pattern and an increased risk of sudden cardiac death. We present this case with the updated literature to emphasise the need to consider the diagnosis of Brugada syndrome in patients admitted to the emergency ward with sudden cardiac arrest. Case Report: A 16-year-old female patient was admitted to the emergency ward with complaints of weakness and abdominal pain, and she had four cardiac arrests during her evaluation period. She was referred to our clinic for permanent pacemaker implantation. She was on a temporary pace maker after having had C-reactive protein. Her physical exam was normal except for bilaterally decreased lung sounds. Lung x-ray and computed tomography, which were performed by another institution, revealed minimal pleural effusion and nothing else of significance. Blood and peritoneal fluid samples were sterile. Echocardiographic exam and cardiac enzymes were also in the normal ranges. Electrocardiographic showed incomplete right branch block in leads V1 and V2. An ajmaline test revealed specific electrocardiographic findings of the type I Brugada pattern. We proposed implanting an implantable cardioverter defibrillator to the patient as there were positive findings on the ajmaline test as well as a history of sudden cardiac arrest. After this treatment proposal, the patient’s family admitted that she had taken a high dose of verapamil and thus, the encountered bradycardia was associated with verapamil overuse. The ajmaline test was repeated as it was contemplated that the previous positive ajmaline test had been associated with verapamil overuse. Implantable cardioverter defibrillator implantation was proposed again as there was a history of sudden cardiac arrest; however, the family did not consent to implantable cardioverter defibrillator, and the patient was discharged and followed up. Conclusion: Brugada syndrome should be considered for patients who are admitted to the emergency ward with sudden cardiac arrest though surface electrocardiographic is normal. If there is a suspicion of Brugada syndrome, repeated electrocardiographic should be performed on different occasions. Diagnosis can be clarified by upper costal electrocardiographic or by administering Na channel blockers during electrocardiographic performance.

Angiocardiography and magnetic resonance imaging to assess pulmonary regurgitation in repaired tetralogy of Fallot

OBJECTIVE This study aimed to compare the results of angiocardiography and cardiovascular magnetic resonance imaging in the assessment of pulmonary regurgitation following repair of tetralogy of Fallot. METHODS We prospectively studied 37 patients with repaired tetralogy of Fallot. After routine examination cardiovascular magnetic resonance imaging (CMR) and cardiac catheterization and angiography were performed. Pulmonary regurgitation (PR) was classified according to the following criteria, using a left lateral angiogram of the main pulmonary artery; insufficiency jet is limited to right ventricular outflow tract (mild); jet reaches the body of right ventricle (moderate); jet fills the apex of the right ventricle (severe). RESULTS Pulmonary regurgitation determined by angiocardiography and CMR was severe in 51.4% and 32.4%, moderate in 27% and 40.5%, and none or mild in 21.6% and 27% of patients respectively. The ability of semi-quantitative estimation of PR determined by cardiac catheterization to distinguish between mild, moderate and severe pulmonary regurgitation was shown to have significant correlation with pulmonary regurgitant fraction obtained by CMR. CONCLUSIONS Angiography obtained during invasive study can be used for the diagnosis and follow-up of pulmonary regurgitation confidently in patients with repaired tetralogy of Fallot and residual pulmonary regurgitation.