Edward V. Colvin

Pulmonary Vein Stenosis After Catheter Ablation of Atrial Fibrillation

BACKGROUND This report describes the complication of pulmonary vein stenosis with resultant severe pulmonary hypertension that developed in 2 patients after successful catheter ablation of chronic atrial fibrillation. METHODS AND RESULTS Three months after successful catheter ablation of atrial fibrillation, both patients developed progressive dyspnea and pulmonary hypertension. Both were found to have severe stenosis of all 4 pulmonary veins near the junction with the left atrium. Balloon dilation of the stenotic pulmonary veins was performed in these patients, with improvement in dyspnea and pulmonary hypertension. CONCLUSIONS The complication of pulmonary vein stenosis is potentially life-threatening, and the application of radiofrequency current within the pulmonary veins with standard catheter technology should be avoided. This complication can be treated with balloon dilation, although the long-term course is unknown.

Balloon dilation of miscellaneous lesions: Results of Valvuloplasty and Angioplasty of Congenital Anomalies Registry

Data on 111 lesions, in addition to pulmonary valve, aortic valve, branch pulmonary stenosis, native coarctation and recoarctation, were submitted to the Valvuloplasty and Angioplasty of Congenital Anomalies Registry. Because there was a very heterogenous group of lesions both in types of lesions and techniques of dilation, only minimal data can be offered concerning the efficacy of the dilation of any or all of these lesions. The registry data do, however, demonstrate the safety of these dilations compared to alternative surgical therapy.

Two-dimensional and color doppler assessment of ventricular septal defect of congenital origin

Two-dimensional echocardiography and color Doppler examinations were performed in 53 patients with 58 ventricular septal defects (VSD) proven surgically or anatomically. All patients also had angiocardiograms. Two-dimensional echocardiography/color Doppler examination detected all VSDs and correctly categorized the site and extension of VSDs in 50 of 58 (86%). All 40 perimembranous VSDs were diagnosed in the left ventricular outflow tract short-axis plane as an area of discontinuity adjacent to septal tricuspid valve leaflet attachment. Fourteen of 16 VSDs with inlet extension showed initial color flow signals along the septal tricuspid leaflet and along the ventricular septum. Of 23 perimembranous VSDs with outlet extension, 19 had flow signals moving directly toward the right ventricular outflow tract. One perimembranous VSD with trabecular extension showed flow signals directed anterolaterally toward the right ventricular free wall. Eleven of 13 muscular VSDs were similarly categorized correctly by color Doppler as inlet, outlet and trabecular. All 5 doubly committed VSDs were correctly diagnosed as an area of discontinuity adjacent to the pulmonary valve in the short-axis view with flow signals directly moving through VSD into right ventricular outflow and pulmonary artery. Angiography correctly detected all VSDs and correctly classified their site and extension in 45 of 58 (77.5%). It misclassified 8 of 40 perimembranous, 3 of 13 muscular and 2 of 5 doubly committed VSDs. Color Doppler compares favorably with angiocardiography in the detection and localization of VSDs.

Early Primary Correction of Tetralogy of Fallot

As a record of achievement, the paper in this issue of The Annuls by Gustafson and colleagues entitled ”Early Primary Repair of Tetralogy of Fallot” is exemplary. There can be no faultfinding with an experience with repair of tetralogy of Fallot in 40 patients in which no deaths have occurred. The effectiveness of the paper in enabling readers (or the authors) to predict the results of the protocol described in future patients or to compare the results with those of other protocols is another matter. Yet these processes are quite necessary to the making of responsible recommendations to future patients and to colleagues. The ”concept” (as the authors put it) of early primary

Etiology and retrieval of retained central venous catheter fragments within the heart and great vessels of infants and children

BACKGROUND The use of centrally positioned venous catheters plays an indispensable role in the care of infants and children. METHODS Since 1992 the authors have seen nine patients who experienced fragmentation and migration of catheter fragments into the central circulation. The patients ranged in age from 6 days to 15 years. RESULTS Sites of migration included pulmonary artery (five patients), superior vena cava (two patients), hepatic vein and innominate vein (one patient). The elapsed time from recognition of retained catheter fragments until retrieval ranged from a few hours to 6 weeks. CONCLUSION All retained fragments were successfully removed during cardiac catheterization without complications.

Prenatally diagnosed hypoplastic left heart syndrome—Outcomes after postnatal surgery

OBJECTIVE To identify prenatally diagnosed cases of hypoplastic left heart syndrome (HLHS) and then to determine postnatal outcomes after surgical interventions. METHODS An ultrasound and pediatric cardiology database was used to identify all fetuses diagnosed prenatally from 1991-1996 with HLHS. Fetal karyotypes were performed on cultured amniocytes. After diagnosis, parents were given several management options: pregnancy termination before 22 weeks, postnatal hospice care, or surgery using the Norwood procedure or cardiac transplantation. Ultrasound and echocardiography findings were later compared to karyotype results and postnatal outcome data. RESULTS Fifteen fetuses with HLHS were identified. Two (16%) chromosome abnormalities and three (20%) structural defects were detected. Three mothers (20%) opted for pregnancy termination, two (13%) chose postnatal hospice care, and one aneuploid fetus had an intrauterine death. Nine parents (60%) chose surgery for their infants; however, one infant was not an appropriate surgical candidate due to a coexisting diaphragmatic hernia. Eight infants underwent surgery and two survived (25%). Of the four infants scheduled to undergo the Norwood procedure, one died preoperatively, two died intraoperatively, and one infant survived and is doing well at age 8 months. Of the four infants scheduled for cardiac transplantation, two died awaiting transplant and one died postoperatively. One infant survived cardiac transplantation but has microcephaly and developmental delay at age two. CONCLUSIONS In prenatally diagnosed HLHS at our institution, the survival rate following surgery for infants felt to be the best candidates was only 25%.

Complete transposition of the great arteries: treatment in the current era.

Transposition of the great arteries usually can be diagnosed by fetal echocardiography, which is important because the arterial switch operation, currently the treatment of choice, must be performed very early in life. The results of the arterial switch operation have been good to date. The dilemma remains, however, whether a fetus in whom a diagnosis of transposition is made should be aborted or treated surgically soon after delivery.

Vegetation biopsy using transesophageal echocardiography guidance: A technique to aid in diagnosis of culture‐negative endocarditis

A method of obtaining a vegetation sample in a culture-negative endocarditis is described. A combination of fluoroscopy and transesophageal echocardiography was utilized to obtain the sample. The results positively influenced the diagnosis and treatment in this 16-yr-old male with complex congenital heart disease.

Systemic effects of intracoronary nitroglycerin during coronary angiography in children after heart transplantation.

Coronary spasm during coronary angiography for vasculopathy in children can be prevented by the intracoronary administration of nitroglycerin. We reviewed the anesthesia and catheterization reports and charts for pediatric transplant recipients who underwent angiography from 2005 through 2010. Correlation analysis was used to study the relation of post-injection systolic blood pressure (SBP) to nitroglycerin dose. Forty-one angiographic evaluations were performed on 25 patients (13 male and 12 female). Mean age was 9.9 ± 3.2 years (range, 3.3-16.1 yr). The mean total dose of nitroglycerin was 2.93 ± 1.60 µg/kg (range, 1-8 µg/kg). There was a significant drop between the baseline SBP (mean, 106 ± 21.6 mmHg) and the lowest mean SBP before nitroglycerin administration (78 ± 13.2, P <0.0001, paired t test). There was no significant additional change in SBP (mean after nitroglycerin administration, 80.7 ± 13.1 mmHg; P = 0.2). There was a significant drop in lowest heart rate between baseline (109 ± 16.5 beats/min) and before nitroglycerin administration (89 ± 14.3 beats/min; P <0.0001, paired t test). There was no significant additional change in heart rate (mean heart rate after nitroglycerin, 84 ± 17.7 beats/min; P = 0.09). There were 2 interventions for SBP before nitroglycerin and 2 after nitroglycerin. One child experienced a transient ST-T-segment change during angiography after nitroglycerin. In the highest dose range, the additional decrease in SBP was 7.2 mmHg (P=0.03). Routine intracoronary nitroglycerin administration in this dose range produced no significant changes in SBP or heart rate in children.

Surgical Management of Unusual Cardiac Tumors in Infants and Children

While most primary tumors of the heart are histologically benign, they are significant space-occupying lesions with serious functional implications for the heart and lungs. Herein, we highlight our experience with the surgical management of selected cardiac tumors in the pediatric population between 2008 and 2010. (1) Intrapericardial teratomas in the fetus can produce fatal tamponade from compression by the attendant pericardial effusion, and a critical life-saving maneuver preoperatively is to drain the effusion prenatally, followed by an expeditious resection after birth. (2) Rhabdomyomas, the most common of the pediatric cardiac tumors, can be intracavitary, large, and associated with the mitral subvalvular apparatus. (3) Cardiac fibromas should be aggressively resected or at least debulked, especially given their propensity for dysrrhythmias. The key to success is as complete a resection as possible, but not at the expense of other normal structures. (4) Complex nonobstructive hypertrophic myopathy can be thought of as a type of neoplastic overgrowth, and aggressive resection of even midcavitary obstructive lesions should be considered as a viable alternative to primary transplantation.