Hypocalcemic rickets: an unusual cause of dilated cardiomyopathy.

We report a breast-fed infant with clinical evidence of rickets and with dilated cardiomyopathy who responded well to supplemental calcium and vitamin D. We believe that this is the first report of such an association in an American child.

Normally connected anomalously draining obstructed pulmonary veins in an infant with mitral atresia: clinical presentation and catheter management.

A patient with hypoplastic left ventricle and double outlet right ventricle underwent pulmonary artery band as a newborn. At age 3 months, cardiac catheterization demonstrated complete closure of his atrial septal defect with decompression of the left atrium via a small levo-cardinal vein. Thus, he had normally connected, anomalously draining obstructed pulmonary veins. He underwent successful catheter intervention with excellent release of the obstruction. This rare finding and technical aspects of catheter intervention are discussed.

Congenital coronary arteriovenous fistula presenting with syncope.

Previous reports of syncope in patients with coronary arteriovenous fistula (CAVF) have theorized that it occurs secondary to a coronary steal phenomenon. We present a case of syncope in a young woman with a CAVF and no anatomic substrate for coronary steal.

Replacement of the mitral valve in an infant with group B streptococcal endocarditis.

Endocarditis due to group B streptococcus is very rare in infants, and may be associated with significant morbidity and mortality. Review of the literature reveals only a single reported case of an infant with this type of streptococcal endocarditis involving the mitral valve. This infant had underlying congenital heart disease, and died shortly after catheterization. We now report group B streptococcal endocarditis occurring in an infant with a structurally normal heart who was treated successfully by replacement of the mitral valve.

Gianturco-Grifka vascular occlusion device for closure of patent ductus arteriosus.

We retrospectively reviewed the results of catheter closure of patent ductus areteriosus using the Ginaturco-Grifka vascular occlusion device in our institution. All patients in whom it was attempted had successful implantation, complete closure on follow-up, and no complications.

Immediate post-operative balloon dilatation of neonatal long segment stenosis of the descending thoracic aorta.

During surgical repair of a neonatal coarctation it was recognized that this was a rare, previously undescribed form of stenosis of the entire descending thoracic aorta. A few hours after the end-to-end surgical repair, the child underwent successful balloon angioplasty, involving the entire descending thoracic aorta, and in which we intentionally avoided the surgical site.

Early experience with the Amplatzer ductal occluder for closure of the persistently patent ductus arteriosus.

Using an Amplatzer duct occluder, 106 patients (weight 21 +/- 18 kg) underwent an attempt at catheter closure of a persistently patent ductus arteriosus (PDA). Their age ranged from 22 days to 48 years. The PDA measured between 1.2 to 8.1 mm at its narrowest diameter. The device was successfully implanted in 105 patients. The immediate closure rate was 70% and gradually increased to 100% at 1-month follow-up. There was no clinical evidence of hemolysis and no incidence of device embolization or bacterial arteritis. Doppler evaluation showed no evidence of aortic arch or pulmonary artery obstruction. The device, which is currently undergoing multicenter clinical trial in the United States, is proving to be a safe and effective device for closure of the persistently PDA.

Postsurgical use of amplatzer septal occluder in cyanotic patients with pulmonary atresia/intact ventricular septum: significance of cor triatriatum dexter and dilated right atrium.

Percutaneous closure of secundum atrial septal defects (ASDs) has been shown to be safe and effective. However, its role after surgery in patients with cyanotic congenital heart disease who may have associated cor triatriatum dexter and a dilated right atrium has not been established. This article reports on successful closure in such patients, including precautions and results.

Closure of a large pulmonary arteriovenous malformation using multiple Gianturco-Grifka vascular occlusion devices.

A large pulmonary arteriovenous malformation was successfully occluded using multiple Gianturco-Grifka vascular occlusion devices. The filler wire protruded from one of the devices and was successfully retrieved 3 weeks after implantation.

Transcatheter coil embolization of abnormal vascular connections using a new type of delivery catheter for enhanced control.

A new type of delivery catheter, designed with a 0.033-inch distal tip that grips a 0.038-inch Gianturco coil, was used to occlude 61 abnormal vascular connections in 44 patients with a complete closure rate of 87%. Withdrawal (n = 6) or repositioning (n = 2) of an inappropriately positioned coil was necessary in 8 of 44 patients, and was successfully achieved in all by the delivery catheter without need for additional equipment.

Branch retinal artery occlusion from a retained left atrial catheter 21 years after operation.

A case of branch retinal artery occlusion due to an embolus from a retained left atrial catheter is presented. Removal was accomplished by reoperation. Prompt removal of any retained intracardiac catheter is recommended.

Use of balloon-expandable stents for coarctation of the aorta: initial results and intermediate-term follow-up.

OBJECTIVES: In this study we report our preliminary results and intermediate-term follow-up (up to 3.5 years) of stent implantation for coarctation of the aorta (COA). BACKGROUND: Balloon angioplasty has gained acceptance as a modality of treatment for COA. Some patients do not respond optimally to balloon angioplasty alone. Balloon-expandable stents have been used in pulmonary arteries and large systemic arteries such as the femoroiliac vessels, with a significant improvement in vessel patency and a reduction in the pressure gradient compared with balloon angioplasty alone. METHODS: Nine patients (>10 years old) with COA in whom balloon dilation alone was thought to be ineffective underwent stent implantation. Seven patients had a previous operation or balloon dilation, or both, to relieve their coarctation but had a significant residual/recurrent gradient. RESULTS: At the time of stent implantation, the systolic and mean gradients decreased from a mean (+/-SEM) of 37 +/- 7 and 14 +/- 3 mm Hg to 4 +/- 1 and 2 +/- 0.6 mm Hg, respectively (p < or = 0.002). The coarctation diameter increased from a mean of 9 +/- 1 to 15 +/- 1 mm (p < 0.002). The patients have been followed for up to 42 months (mean 18, median 13) with no complications; the stents remain in position with no fracture. One patient underwent further successful dilation 3 years after stent implantation because of an exercise-induced gradient. No other intervention has been required. The systolic gradient at latest follow-up is 7 +/- 2 mm Hg. Only two (a 44-year old with diabetes and a 50-year old with long-standing hypertension) of five patients previously requiring antihypertensive treatment still remain on medications for blood pressure control. CONCLUSIONS: The use of stents in COA is a feasible alternative to surgical repair or balloon angioplasty in selected patients with an effective gradient reduction. Intermediate-term follow-up shows excellent gradient relief, with no complications in this group of patients.

Anomalous origin of left coronary from right pulmonary artery in hypoplastic left heart syndrome.

An infant with hypoplastic left heart syndrome presented for surgical repair at 9 months of age, the ductus having remained open in the presence of a restrictive atrial septal defect. In addition, an anomalous left coronary artery originating from the right pulmonary artery was found. After preliminary blade/balloon atrial septostomy, a successful modified Norwood procedure with concomitant reimplantation of the anomalous coronary artery was performed.

Doppler echocardiographic evaluation of pulmonary vascular resistance in children with congenital heart disease.

Noninvasive assessment of pulmonary vascular resistance has not been well defined. Cardiac catheterization findings in 33 patients with congenital heart disease (mean age 1.4 years) were compared with Doppler echocardiographic parameters. The right ventricular pre-ejection period (RVPEP), ejection time (RVET), and the ration RVPEP/RVET correlated better with pulmonary vascular resistance than with pulmonary artery pressure. A highly significant correlation with a small standard error of estimate (SEE) was demonstrated between pulmonary vascular resistance and a newly derived parameter RVPEP/velocity time integral (VTI) [r = 0.87, p < 0.0001, SEE = 2]. An RVPEP/VTI value of < 0.4 seconds/meter (M) was able to select patients with pulmonary vascular resistance < 3 Wood Unit.M2, even in the presence of pulmonary artery hypertension caused by increased pulmonary blood flow, with 97% accuracy (100% sensitivity, and 92% specificity). An RVPEP/VTI value of 0.4 to 0.6 seconds/M identified patients with pulmonary vascular resistance between 3 to 7.5 Wood Unit.M2 with 91% accuracy, and a value of > or = 0.6 seconds/ M selected patients with total pulmonary vascular resistance > or = 7.5 Wood Unit.M2 with 94% accuracy.

Periodic paralysis and ventricular tachycardia: possible role of calcium channel blockers.

Few patients have been reported with familial periodic paralysis and ventricular tachycardia. The natural history of these cases was unfavorable, with most dying because of a presumed cardiac dysrhythmia. We report for the first time the results of an electrophysiologic study of a similar case and the successful use of calcium channel blockers, shedding light on the possible mechanism and management of these patients.

Hyperkalemic periodic paralysis with cardiac dysrhythmia: a novel sodium channel mutation?

A patient is presented with hyperkalemic periodic paralysis (HyperPP) and a cardiac dysrhythmia. An amino acid substitution (Val783Ile) in the adult skeletal muscle sodium channel gene was detected. Although lack of available family members precluded rigorous genetic tests, the sodium channel change may be responsible for HyperPP in this patient and could also be responsible for the associated cardiac dysrhythmia.