Early and late presentation of coronary artery fistula: a possible natural progression?

We present two patients, one 10 years old and another 43 years old, who both had successful transcatheter closure of left main coronary artery to right atrium fistulas. The older patient had a larger fistula as well as more symptoms and a complicated post-procedure course. Closure of medium or large coronary artery fistulas should be considered at younger ages to minimise future complications.

Paternal fibrillin-1 mutation transmitted to an affected son with neonatal marfan syndrome: the importance of early recognition.

We describe a case of neonatal Marfan syndrome diagnosed because of a family history, dysmorphic features, and cardiac abnormality. The echocardiogram showed aortic root dilatation. Molecular genetic studies showed a mutation in exon 31 of the FBN1 gene in the infant and father. The infant was treated with losartan, which significantly slowed the rate of enlargement of the aorta.

Transcatheter device closure of atrial septal defects in patients older than 60 years of age: immediate and follow-up results.

UNLABELLED: Atrial septal defect (ASD) closure reduces symptoms and prevents ongoing congestive heart failure. However, little is known about device closure in the elderly (age > 60 years) and whether it is a safe and effective treatment. In this study, we report our results with ASD transcatheter closure in the elderly patient > 60 years of age using the Amplatzer Septal Occluder (ASO) device. METHODS: Between September 1999 and March 2007, a total of 41 patients (24 females and 17 males) who were > 60 years of age (range 62-87.2 years) and had indications for ASD closure (right ventricular enlargement, clinically symptomatic dyspnea, fatigue, palpitations, exercise intolerance, transient ischemic attacks and/or stroke) underwent an attempt of transcatheter device closure using the ASO. RESULTS: The median Qp/Qs ratio was 2.3 (range 1-7.5). The median mean pulmonary artery pressure was 26 mmHg (range 11-52 mmHg). The median size of the ASD as measured by intracardiac echocardiography (ICE) (n = 38) or transesophageal echocardiography (TEE) (n = 3) was 18.9 mm (range 8-40 mm), and the median balloon-stretched (stop-flow technique) diameter (n = 32) was 23.5 mm (range 12-40 mm). The procedure was successful in all patients. 44 devices were deployed in the 41 patients (3 patients received 2 devices each). A 6 mm fenestration was created in a 30 mm device that was placed in 2 patients with left ventricle diastolic dysfunction. The median fluoroscopy time was 10 minutes (range 4-24.8 minutes), and the median procedure time was 60 minutes (range 26-110 minutes). Complications encountered during or within 24 hours after the procedure included: hematoma at the site of catheter insertion in 4 patients, small pericardial effusion in 5, and in 1 patient, the pacemaker lead was dislodged requiring reimplantation. The complete closure rate as assessed by ICE or TEE immediately after the procedure was 82%. However, complete closure, as assessed by transthoracic echocardiography at 24 hours and 6-24 months postintervention, was 82% and 97%, respectively. Two patients did not return for follow up and 4 patients were known to have expired for reasons unrelated to their ASD closure. At a median interval of 6 months after closure, the right ventricular end-diastolic dimension decreased from 38.9 +/- 9 mm preprocedure to 26.6 +/- 7 mm (p < 0.001; n = 31) postprocedure, and 89% of the patients showed improvement in symptoms. On a median follow-up interval of 28 months (range 6-79 months), 1 patient was noted to have a femoral pseudoaneurysm. Patients were doing well with no major clinical events. CONCLUSION: In patients older than 60 years of age, device closure of ASDs is safe and an effective alternative to surgery. Right-heart remodeling can occur in patients > 60 years of age.

Modest maternal caffeine exposure affects developing embryonic cardiovascular function and growth.

Caffeine consumption during pregnancy is reported to increase the risk of in utero growth restriction and spontaneous abortion. In the present study, we tested the hypothesis that modest maternal caffeine exposure affects in utero developing embryonic cardiovascular (CV) function and growth without altering maternal hemodynamics. Caffeine (10 mg.kg(-1).day(-1) subcutaneous) was administered daily to pregnant CD-1 mice from embryonic days (EDs) 9.5 to 18.5 of a 21-day gestation. We assessed maternal and embryonic CV function at baseline and at peak maternal serum caffeine concentration using high-resolution echocardiography on EDs 9.5, 11.5, 13.5, and 18.5. Maternal caffeine exposure did not influence maternal body weight gain, maternal CV function, or embryo resorption. However, crown-rump length and body weight were reduced in maternal caffeine treated embryos by ED 18.5 (P < 0.05). At peak maternal serum caffeine concentration, embryonic carotid artery, dorsal aorta, and umbilical artery flows transiently decreased from baseline at ED 11.5 (P < 0.05). By ED 13.5, embryonic aortic and umbilical artery flows were insensitive to the peak maternal caffeine concentration; however, the carotid artery flow remained affected. By ED 18.5, baseline embryonic carotid artery flow increased and descending aortic flow decreased versus non-caffeine-exposed embryos. Maternal treatment with the adenosine A(2A) receptor inhibitor reproduced the embryonic hemodynamic effects of maternal caffeine exposure. Adenosine A(2A) receptor gene expression levels of ED 11.5 embryo and ED 18.5 uterus were decreased. Results suggest that modest maternal caffeine exposure has adverse effects on developing embryonic CV function and growth, possibly mediated via adenosine A(2A) receptor blockade.

Percutaneous closure of left ventricular pseudoaneurysms after Ross procedure.

Pseudoaneurysm formation is a dangerous, but uncommon, complication after the Ross procedure. We describe a patient in whom two pseudoaneurysms formed at the proximal suture line of the pulmonary autograft. These were successfully treated percutaneously, one by placement of an Amplatzer Septal Occluder (AGA Medical, Golden Valley, MN), and the other with coil embolization.

Intentional avulsion fracture of the second cervical vertebra in a hypotonic child.

Children uncommonly present with cervical fractures due to high-impact injuries. A 4-year-old child with developmental delay and chronic hypotonia presented to the hospital with acute onset of irritability and transient difficulty in walking. Lateral neck x-rays showed avulsion fracture of the second cervical vertebra. Neck magnetic resonance imaging study showed injury to the interspinal ligaments between the first and second cervical vertebrae in addition to confirming the acute avulsion fracture of C-2. The child was injured after he was thrown onto a bed from a distance of 1 to 2 ft according to his babysitter's admission. This is the first reported pediatric case presenting this type of inflicted fracture in a hypotonic child, although hypotonia may not have played a causal role.

Thoracic sequels after thoracotomies in children with congenital cardiac disease.

The standard surgical approach for closed heart procedures in small infants and children is to use a posterolateral thoracotomy incision, which results in the division of the latissimus dorsi and serratus anterior muscles. The aim of our study was to determine the frequency and type of musculoskeletal deformities in children undergoing surgery with this approach for congenital cardiac disease. We included 49 children, 28 boys and 21 girls, in the study. Their mean age was 10.2 +/- 4.8 years, the mean age at the time of surgery was 3.8 +/- 4.0 years, and they were evaluated at an average of 6 years after the thoracotomy. Of the patients, 94% had various musculoskeletal deformities. Scoliosis was observed in 15 patients (31%) but only in two patients did the curves exceed 25 degrees. Of these patients, three-fifths had aortic coarctation. Elevation of the shoulder was seen in 61%, winged scapula in 77%; while 14% had asymmetry of the thoracic wall due to the atrophy of the serratus anterior muscle. Deformity of the thoracic cage was observed in 18%; and 63% had asymmetry of the nipples. Thus, we found that musculoskeletal deformities are frequent after thoracotomies in children with congenital cardiac disease. Patients who have undergone such procedures for cardiac or noncardiac surgery should be followed until their skeletal maturation is complete. Techniques sparing the serratus anterior and latissimus dorsi muscles should be preferred. These adverse effects of thoracotomy may be another reason for using interventional procedures in these cases.

Non-invasive evaluation of aortic regurgitation by Doppler echocardiography in children: comparison with contrast angiography.

Doppler indexes have been used successfully to determine the severity of aortic regurgitation (AR) in adults but have not been evaluated in children. To determine the accuracy of pulsed, color, and continuous-wave (CW) Doppler echocardiographic indexes in assessing the degree of AR in children, the correlation between the non-invasive measurements and angiographic grading of the regurgitant flow (1+ to 4+) was examined in 14 children (mean age 11 +/- 3 years) with chronic AR. Forward and reverse flows in the aortic arch were evaluated from the suprasternal notch using pulsed Doppler. Aortic time-velocity integrals (TVI) were measured during systole (forward flow) and diastole (reverse flow), and the ratio of reverse to forward TVI (%) was calculated. Doppler color flow mapping was used to detect and assess the severity of AR (which appears as mosaic turbulent signals extending in the left ventricular outflow tract during diastole) by using four color Doppler grades of severity. The envelope of the flow velocity pattern in diastole was recorded from the CW Doppler signal of AR with the transducer in the lower sternal border to determine the peak flow velocity and deceleration slope (alpha) indexes. The ratio of reverse to forward aortic TVI and color flow mapping grading showed strong correlation with angiographic grade (r=0.92 and r=0.86, respectively) but AR slope (alpha) and peak flow velocity did not correlate well with the angiographic grade (r=-0.039 and r=0.74, respectively). We concluded that the severity of AR in children as determined by angiographic grading can be estimated with reasonable accuracy by noninvasive technique based on color and pulsed wave Doppler. Use of these indexes may obviate the need for angiography to detect the severity of AR in children.

Noncompaction of ventricular myocardium: a study of twelve patients.

We report 12 patients with ventricular noncompaction who were echocardiographically identified at our institution since 1991. The mean age at presentation was 3.5 years. Five patients had isolated noncompaction. Three of them had subnormal left ventricular systolic function at presentation. Noncompaction was associated with complex congenital heart defect in 3 patients. Four patients had simple congenital heart defects: pulmonary stenosis, coarctation of aorta with aberrant origin of right subclavian artery, ventricular septal defect, and partial anomalous pulmonary venous return. The observed rhythm abnormalities were Wolff-Parkinson-White syndrome and paroxysmal supraventricular tachycardia, bigemini ventricular extrasystoles, and left bundle branch block. A transvenous pacemaker was implanted in a patient because of complete heart block. Noncompaction of the ventricular myocardium is rare. Our patients clearly represent the clinical and morphological spectrum of this disorder. Distinct morphological features can be diagnosed on 2-dimensional echocardiography.

Surgical outcome of congenital valvar aortic stenosis.

Valvar aortic stenosis is a common congenital heart defect for which surgical procedures can be done with low risk except in infants whose conditions are seriously compromised. The purpose of this report was to present our experience with the results of surgical aortic valvotomy for congenital valvar aortic stenosis performed at our hospital. The study group consisted of 24 patients, 3 females and 21 males, with ages ranging from 1 to 15.5 years (mean age 8.5 years), who underwent aortic valvotomy for valvar aortic stenosis. The case records of all the patients were retrospectively reviewed. They were followed for 1-10.5 years (mean 5.02+/-2.38 years) after relief of aortic stenosis, and were scheduled for reevaluation. Sixteen of the 24 patients were recatheterized and 15 (93.7%) were found to have aortic regurgitation on angiography. Peak systolic pressure gradients (mean+/-SD) were 65.9+/-19.5 mmHg before and 36.7+/-14.8 mmHg (p<0.05) after the operatilon. Of the 24 patients, 45.8% had a new postoperative diastolic murmur. Twenty patients (83.3%) had residual stenosis and three (12.5%) had recurrent stenosis. Two patients (8.3%) had undergone reoperation six to seven years after the initial aortic valvotomy, and most of the others will require reoperation in the future. No sudden deaths occurred in this series. Timely relief of obstruction prevents sudden death and produces symptomatic improvement in valvar aortic stenosis, but aortic valvotomy is only a palliative measure.

Anomalous left coronary artery from the main pulmonary trunk: physiologic and clinical importance of its association with patent ductus arteriosus and pulmonary hypertension.

Origin of the left main coronary artery from the pulmonary trunk is an extremely rare, fatal, but potentially treatable anomaly. Left ventricular perfusion with desaturated blood with low pressure from the pulmonary artery results in early death. Associated pulmonary hypertension can help to prevent ischemia. We present a four-month-old boy with this anomaly associated with patent ductus arteriosus and pulmonary hypertension.

Influence of D-net (EUROPEAN GSM-standard) cellular telephones on implanted pacemakers in children.

This study was designed to evaluate possible interactions between digital cellular telephones and implanted pacemakers in children. The study comprised 95 patients (53 males and 42 females) with a mean age of 11.5 +/- 4.6 years (range 1-22 years). The average time from pacemaker implantation was 2.5 years (range 1 month-12 years). Fourteen (15%) devices were dual chamber and the remaining were single chamber pacemakers. The following companies manufactured the pacemakers tested: Medtronic (n = 42), Telectronics (n = 9), Vitatron (n = 16), Pacesetter (n = 19), CPI (n = 8), and Biotronik (n = 1). All the patients were tested in the supine position during continuous ECG monitoring. After completion of the routine pacemaker check, the effects of the European Global system for mobile communication (GSM) was tested using two cellular telephone models (Ericsson GA 628 and Siemens S 25, 2-W power). For this purpose, atrial and ventricular sensitivity settings were programmed to the most sensitive values, and the tests were carried out in the unipolar and bipolar sensing modes. The evaluation was performed during ringing, switching on/off, and conversation phase with the cellular telephone positioned over the pulse generator and around the pacemaker pocket. A malfunction of the pacemaker was not observed in any patient. Only 1 (1%) of 95 patients showed a brief oversensing problem during calls with the cellular telephone. In this case, an AAIR pacemaker was implanted transvenously in a subcutaneous pocket and the sensing defect occurred only with the unipolar sensing mode and was not reproducible. Once the source of interference was removed, no sensing defect was detected and the patient remained asymptomatic. No symptoms were experienced in this study. The authors believe that pacemaker dependent patients with nonprotected pulse generators manufactured at the beginning of 1990s may be tested by their physicians for possible interferences before they use a digital cellular telephone.

Visceroatrial situs solitus with atrioventricular alignment discordance double outlet right ventricle and superoinferior ventricles: fetal and neonatal echocardiographic findings.

Visceroatrial situs, ventricular morphology, and atrioventricular and ventriculoarterial alignments can reliably be assessed with fetal echocardiography. We herein describe, to our knowledge, the first reported fetal echocardiographic diagnosis of a fetus with visceroatrial situs solitus, atrioventricular alignment discordance, double outlet right ventricle, and superoinferior ventricles of a 22-year-old gravida III para II woman with a previous history of intrauterine fetal death. She had been evaluated at another center and was referred to our unit because of the suspicion about her diagnosis and underwent fetal ultrasonographic evaluation at 39 weeks gestation. Neonatal echocardiographic evaluation and autopsy findings confirmed the diagnosis. Accordingly there was (solitus, L-loop ventricle, and anterior aorta) double outlet right ventricle, superoinferior ventricles with diminutive right ventricle, noncommitted trabecular ventricular septal defect, and pulmonary hypertension.