Total correction in tetralogy of Fallot with anomalous major coronary artery: an alternative method to conduit use.

INTRODUCTION: A coronary artery anomaly precludes the use of a trans-annular patch in right ventricular outflow tract (RVOT) reconstruction. Herein we present three patients with coronary artery anomalies who underwent total corrective operations without using a conduit. METHODS: Between 2007 and 2010, 84 patients with tetralogy of Fallot (TOF) were operated on. Nine (9.4%) of them had a coronary artery anomaly. Three (3.1%) of the patients were operated on using the double-outflow technique and two had a Blalock-Taussig shunt before the total corrective operation. In two patients, the left anterior descending artery (LAD) and in one, the right coronary artery (RCA) crossed the RVOT. RESULTS: Postoperatively, the right-to-left ventricular pressure ratios were 0.45, 0.59 and 0.60 after cardiopulmonary bypass. No gradient was detected in the RVOT in postoperative echocardiographical measurements (< 15 mmHg gradient). In all three patients, there were moderate pulmonary insufficiencies. All were discharged home on the sixth day postoperatively. Mean follow-up duration was 9.8 ± 8 months. In the follow up of all three patients, there were moderate pulmonary insufficienciencies but no right ventricular dysfunction. CONCLUSION: The 'double-outflow' technique is appropriate for TOF patients with a major coronary artery anomaly since it can easily be performed without the need of a conduit.

Giant right atrial myxoma leading to cardiac arrest in an infant.

We present the case of a three-month-old infant with a giant right atrial myxoma obstructing the tricuspid valve, who following haemodynamic deterioration and cardiac arrest, was operated upon as an emergency. On echocardiogram, there was a mass attached to the tricuspid annulus, in close proximity to the septal leaflet, with dimensions of 16.6 × 12.5 mm. The mass was prolapsing through the tricuspid valve into the right ventricle and obstructing the inflow. While preparing for surgery, cardiac arrest occurred, so the patient underwent an emergency operation under cardiopulmonary resuscitation. The mass was excised without damaging the tricuspid valve and the conduction system. Histologically, the mass consisted of a myxoid matrix with scatted globoid and star-shaped myxoma cells. The patient stayed 15 days in the intensive care unit and was discharged home on the 20th day postoperatively. Although accepted as a benign tumour, a myxoma can display an aggressive clinical course in infants. In centres where cardiac operations cannot be performed, these patients need to be transferred to cardiac centres as soon as possible. Whatever the clinical presentation, we advocate immediate surgical extirpation of the tumour in order to avoid any unpredictable consequences in its clinical course.

Increased QT dispersion in breath-holding spells.

AIM: Breath-holding spells are common in infancy and early childhood, and patients are frequently referred to paediatric cardiology clinics for exclusion of heart disease. Recent data reveal subsequent development of epilepsy and neurocardiogenic syncope. Autonomic dysregulation and increased vagal stimulation leading to cardiac arrest and cerebral ischaemia is considered as the cause. Iron deficiency anaemia may be associated with these spells. We studied QT dispersion for the assessment of ventricular repolarization in these patients. METHODS: The study group consisted of 19 girls and 24 boys between 3 and 108 mo of age (mean +/- SD = 22.7 +/- 17.7 mo); and the control group consisted of 13 girls and 12 boys between 3 and 57 mo of age (mean +/- SD = 22.9 +/- 15.1 mo). QT interval was measured; corrected QT interval (QTc), QT dispersion (QTd) and QTc dispersion (QTcd) were calculated from 12-lead surface electrocardiograms of the patients and the control group. RESULTS: There was no statistically significant difference in terms of QT and QTc intervals between patient and control groups, while QTd and QTcd values were significantly increased in patients with breath-holding spells compared to the healthy children. QT dispersion was 59.5 +/- 35.9 ms and 44.8 +/- 11.9 ms, respectively, in patients and controls (p < 0.05). QTc dispersion was 102.1 +/- 41.9 ms and 79.6 +/- 24.6 ms, respectively (p < 0.01). The presence of iron deficiency did not effect the QT and QTc dispersion. CONCLUSION: QT dispersion is increased in patients with breath-holding spells, and this finding justifies further investigation for rhythm abnormalities and autonomic dysfunction in this patient group.

A case of superoinferior ventricular heart with situs ambiguus, dextroventricular loop, and levo transposition of the arteries: prenatal and postnatal echocardiographic diagnosis.

A unique case of superoinferior ventricles, left atrial isomerism, concordant atrioventricular connection, and discordant ventriculoarterial connection is described. The associated anomalies were azygous vein continuation, large subpulmonary and apical ventricular septal defects, and left ventricular outflow tract obstruction (pulmonary stenosis). The diagnosis was mostly made by fetal echocardiography and the superoinferior location of the ventricles was confirmed by postnatal echocardiography. Fetal and postnatal echocardiography facilitated the precise morphologic diagnosis and segmental analysis of this defect.

Sotalol in treatment of pediatric cardiac arrhythmias.

BACKGROUND: There is limited experience on sotalol use in the management of childhood arrhythmias. This study reviews the results of our experience with oral sotalol for treatment and prevention of tachyarrhythmias in children. METHODS: The records of 62 patients (27 female, 35 male, mean age: 8.5+/-5.3 years) treated with sotalol for supraventricular or ventricular arrhythmias from 1994 to 1999 at our institution were reviewed. Demographic, clinical, echocardiographic, electrocardiographic (ECG), ambulatory ECG and electrophysiologic variables were collected. RESULTS: Forty-two (63.6%) patients had re-entrant supraventricular tachycardia, eight patients (12.9%) had atrial tachycardia, one patient (1.6%) had junctional ectopic tachycardia, four patients (6.5%) had ventricular tachycardia, and seven patients (11.3%) had complex ventricular arrhythmias, as evidenced by surface or ambulatory ECG records; or revealed during the electrophysiological study. The mean sotalol dose was 3.9+/-1.2 mg/kg per day. In 15.5+/-13.9 months of sotalol use 50% (n=31) had complete relief of symptoms and/or arrhythmia and 29% (n=18) had partial relief. Sotalol was ineffective in 20% (n=13). Sotalol was more effective in re-entrant type supraventricular tachycardias (P=0.012). Sotalol was the first choice in 35.5% of patients. The sotalol therapy was initiated in inpatient settings in 40.3% (25 patients). Complications due to sotalol were seen in six patients (five patients developed bradycardia/pauses, and one patient had torsades de pointes) for which the sotalol dose was modified. In patients with sick sinus syndrome, a pacemaker was implanted and in another patient sotalol was stopped. CONCLUSION: Sotalol, being an effective and safe drug particularly in children, is a good therapeutic alternative for the preventive treatment of childhood tachyarrhythmias.

A late complication with the CardioSEAL ASD occluder device and need for surgical revision.

A late complication of the CardioSEAL atrial septal defect (ASD) occluder is reported. Although left atrial umbrella was completely epithelialized and occluded ASD without residual defect, the right atrial umbrella protruded toward the center of right atrium after 18 months. We believe this may be associated with the structural abnormality of the device.

Preliminary pediatric transesophageal echocardiography experiences.

With transesophageal echocardiography (TEE), a new echocardiographic window is obtained which enables cardiologists to explore the heart from the esophagus and stomach. However, the procedure, when first undertaken, may present certain difficulties for the cardiologist in interpreting the anatomical findings and approaching a diagnosis. We thus convey our first experiences and results of TEE in 107 pediatric patients. Transesophageal echocardiography (TEE) was performed in 107 pediatric patients at our institution between December 1998-February 2001, using the standard techniques and following the standard criteria suggested by the American heart Association. The mean age of 54 male (50.5%) and 53 female (49.5%) patients was 7.8 years. Intubation difficulty was experienced in four cases. In one case, while drawing the transducer back from the esophagus, it kinked at the hypopharynx. None of the cases had major hemorrhage or esophageal rupture, and only a few cases had minor pharyngeal injuries or hemorrhages. We used TEE in detecting vegetations in patients with possible endocarditis, and evaluating the prosthetic valves and abnormal pulmonary venous return. We also used TEE to clarify preooperative anatomical details, postoperative complications and residual defects of complex congenital cardiac anomalies. Transcattheter closure of 47 secundum atrial septal defects (ASD)'s and a muscular ventricular septal defect (VSD) (both during patient selection and during the procedure) were accomplished under TEE guidance. As the pediatric cardiologists gain more experience in performing TEE, this technique will have a wider and more effective use in the pediatric population.

Can subclinical valvitis detected by echocardiography be accepted as evidence of carditis in the diagnosis of acute rheumatic fever?

AIM: Subclinical valvar insufficiency, or valvitis, has recently been identified using Doppler echocardiography in cases of acute rheumatic fever with isolated arthritis or chorea. The prognosis of such patients with acute rheumatic fever and subclinical valvitis is critical when determining the duration of antibiotic prophylaxis. We aimed, therefore, prospectively to investigate the association of silent valvitis in patients having rheumatic fever in the absence of clinical evidence of cardiac involvement, and to evaluate its prognosis. METHODS AND RESULTS: Between November 1998 and September 1999, we identified 26 consecutive patients with silent valvitis in presence of rheumatic fever but in the absence of clinical signs of carditis. The patients, eight female and 18 male, were aged from 6 to 16 years, with a mean of 9.9+/-2.7 years. Major findings were arthritis in 16, chorea in 7, and arthritis and erythema marginatum in 1 patient. Two cases had arthralgia with equivocal arthritic signs and Doppler echocardiographic findings of pathologic mitral regurgitation. Silent pathologic mitral regurgitation was found in 12 cases, and aortic regurgitation in 2 cases. All patients with arthritic findings were treated with acetylsalicylic acid with one exception, this patient receiving both prednisone and acetylsalicylic acid. No antiinflammatory treatment was given to patients with chorea. After a mean follow-up of 4.52 months, valvar regurgitation disappeared in 4 patients, including the one with migratory arthralgia and no other major criterions. All six patients with chorea and silent carditis still have mitral insufficiency. CONCLUSION: Acute rheumatic fever without clinical carditis is not a benign entity. Doppler echocardiographic findings of subclinical valvar insufficiency, therefore, should be considered as carditis when seeking to establish the diagnosis of acute rheumatic fever.

Transcatheter closure of secundum atrial septal defects, a ventricular septal defect, and a patent arterial duct.

We report our clinical experience with the newly developed Amplatzer device in transcatheter closure of nine atrial septal defects (ASDs), one ventricular septal defect (VSD), and one patent arterial duct (PDA). Eleven patients with ASD (age range 2.5-18 years) selected according to the location and size of the defect by transesophageal echocardiography (TEE), a five-year-old patient with muscular VSD and a one-year-old patient with PDA were considered for transcatheter closure with Amplatzer devices. All procedures were performed under general anesthesia with fluoroscopic and TEE guidance, following a routine hemodynamic evaluation in the catheter laboratory. The optimal device size was selected after the balloon sizing of the ASDs. The sizes of the VSD and PDA were measured on TEE and angiography. The patients were discharged at 24 hours, after an evaluation with x-ray, electrocardiogram (ECG), and echocardiography; they were on 3-5 mg/kg/day aspirin and infective endocarditis prophylaxis for six months after the procedure. They were reassessed at six to eight weeks and Holter monitoring was done in addition. Devices were used for nine ASD patients, and for the VSD and the PDA patients. Mean ASD size was 14.3 +/- 5.3 mm at TEE and 18.3 +/- 4.3 mm at balloon sizing (p=0.02). The mean size of the device was 18.7 +/- 4.2 mm. The procedure time and the fluoroscopy time were 46.1 +/- 12.3 and 12.9 +/- 1.6 minutes, respectively. Immediately after the procedure, four patients (44%) had trivial shunts (TS). TS remained in only two during discharge, and no shunt was observed at second evaluation. The devices were similarly applied to VSD (12-7 mm) and PDA (8-6 mm) patients. Both cases had TS immediately, which disappeared at 24 hours. None of the patients had major complications. Junctional rhythm developed in one patient, and another patient had frequent supraventricular extrasystoles. Amplatzer is an effective and safe device for transcatheter closure of ASD, VSD, or PDA, especially in pediatric patients.

Analysis of 2017 Holter records in pediatric patients.

Holter monitoring (HM) is widely used in arrhythmic disorders of adult patients; however, studies in the pediatric age group are limited. This study aims to determine the value of HM in diagnosis and treatment of disorders related to arrhythmias in pediatric patients. We examined 2,017 Holter records of 1,500 children who applied to our institution between November 1994 and October 1998. The age ranged from 0-24 years (52% male, 48% female). The indications for HM were screening for arrhythmic symptoms (palpitation, chest pain, syncope) in 67 percent, monitoring dysrhythmic therapy in 17 percent, postoperative control in five percent, and pacemaker control in four percent. Palpitation is the leading presenting symptom, with more frequent findings of supraventricular extrasystole (SVE), supraventricular tachycardia (SVT), ventricular extrasystole (VE) and complete heart block (CHB) when compared to other symptoms. Only 5.3 percent of the patients had arrhythmic symptoms during monitoring and asymptomatic patients had more frequent arrhythmias. SVT, VE, and CHB are more frequent findings in the abnormal heart with previous cardiac operations. The diagnostic yield is low with arrhythmic symptoms in the pediatric age group; however, HM enables cumulative evaluation of heart rhythm and rhythm variability, which is important in diagnosing silent arrhythmias in high risk groups (abnormal heart, postoperative heart).

Dilatation of a restrictive interatrial communication using a balloon angioplasty catheter.

Balloon atrioseptostomy is a life-saving procedure palliating certain congenital heart defects like transposition of the great arteries, right or left atrioventricular valve atresia, hypoplastic left heart syndrome, and pulmonary hypertension. Occasionally the Rashkind balloon septostomy technique may be ineffective in creating an adequate interatrial communication. We performed balloon dilatation of a restricted atrial septal defect using a balloon angioplasty catheter in a three-month-old infant.