Gary F. Sholler

Echocardiographic assessment of aortic root dimensions in normal children based on measurement of a new ratio of aortic size independent of growth

Two-dimensional echocardiography is commonly used as a method of monitoring aortic root dimensions in children with connective tissue disease. Measurements are usually standardized to body surface area (BSA) to account for growth. However, there are several theoretical and practical disadvantages to adopting this approach, and there has been little investigation of alternative methods of standardization. This study of 48 normal children and adolescents (age range 2 weeks to 23 years) was performed to determine the relation of 2-dimensional echocardiographic aortic root dimensions to indexes of body size and growth, and to examine a simple means of internally standardizing aortic root measurements to create an index of aortic root size independent of growth. Maximal diameters in the parasternal long-axis view were recorded at 4 levels: annulus, sinuses of Valsalva (SOV), supraaortic ridge (SAR), and ascending aorta (AAO). Ratios of aortic root size were created by internally standardizing aortic root diameters to aortic annular size. All diameters correlated closely with age, height, weight, and BSA (all r > 0.87). Linear regression in each case showed a significant positive slope (all p < 0.0001). The best predictor of aortic dimensions was height, with r values of 0.93 for annulus, SOV, and AAO, and 0.95 for SAR. Ratios of SOV/annulus, SAR/annulus, and AAO/annulus remained constant, with no correlation with age or any growth parameters. Mean values and 95% confidence limits were: SOV/annulus 1.37 (1.18-1.56); SAR/annulus 1.11 (0.95-1.28); and AAO/annulus 1.16 (0.97-1.35). Standardization to height, or the use of internally standardized aortic root ratios, provides a simple and accurate alternative to standardization to BSA for assessing aortic root dimensions in normal growing children.

Velo-cardio-facial and partial DiGeorge phenotype in a child with interstitial deletion at 10p13--implications for cytogenetics and molecular biology.

We report on a female with a interstitial deletion of 10p13 and a phenotype similar to that seen with the 22q deletion syndromes (DiGeorge/velo-cardio-facial). She had a posterior cleft palate, perimembranous ventricular septal defect, dyscoordinate swallowing, T-cell subset abnormalities, small ears, maxillary and mandibular hypoplasia, broad nasal bridge, deficient alae nasi, contractures of fingers and developmental delay. This could indicate homology of some developmental genes at 22q and 10p so that patients with the velocardiofacial phenotype who do not prove to be deleted on 22q are candidates for a 10p deletion.

Primary intraventricular cardiac tumors in children: contemporary diagnostic and management options.

SummaryWe reviewed the intraventricular cardiac tumors presenting at our institution between 1985–1991, studying the presentation, modes of investigation, and evidence of hemodynamic compromise. Thirteen patients presented with intraventricular tumors during the study period. Two of the tumors were rhabdomyosarcomas, one was a myxoma, and 10 were rhabdomyomas. All patients were evaluated with two-dimensional and pulsed Doppler echocardiography and B-color imaging was undertaken in three patients. Four patients presented for elective scans to complement investigations for tuberous sclerosis, seven patients had cardiac symptoms, and two patients presented prenatally. Obstruction to intracardiac flow was present in five patients. Two patients had the tumor excised and one had an open biopsy of the tumor. One patient had an transvascular biopsy at cardiac catheter. Early detection of cardiac tumors is increasing, particularly rhabdomyomas. With fetal echocardiography, more patients should come to attention prenatally. B-color may be a useful addition in assessing cardiac tumors, aiding detection and definition of intramural lesions.

Minimally invasive management of transposition of the great arteries in the newborn period

This study reports on a predominantly noninvasive management program for neonatal transposition of the great arteries [TGA] incorporating balloon atrial septostomy [BAS] under echocardiographic control. BAS was performed in 25 consecutive patients presenting with TGA between April 1988 and April 1990. Structural and coronary anatomy was evaluated echocardiographically with angiographic supplementation only when additional data were required. This information was correlated, where possible, with direct anatomic findings and subsequent course. BAS was performed through the umbilicus in 17 patients (85% of patients in whom this approach was attempted). Thirteen patients did not require ventilation during BAS. There were minimal complications and satisfactory septostomies in all cases. Coronary anatomy was correctly predicted in all patients where anatomic correlation was available. Without invasive investigation 9 patients underwent neonatal arterial switch procedures and 2 underwent palliative procedures. BAS under echocardiographic control proved safe, effective, minimally traumatic and mostly possible via the umbilical vein. The umbilical vein, where patent, permitted rapid safe access for BAS. Echocardiographic diagnosis of the coronary artery, and structural and functional anatomy was reliable and allowed minimally invasive preoperative management in many patients.

Branch pulmonary artery growth after blalock-taussig shunts in tetralogy of fallot and pulmonary atresia with ventricular septal defect: a retrospective, echocardiographic study.

Abstract. We performed a retrospective echocardiographic study in tetralogy of Fallot (TOF) or pulmonay atresia with ventricular septal defect (PA&VSD) to evaluate the effects of Blalock–Taussig shunt on branch pulmonary artery growth. There were 35 patients with TOF and 11 with PA&VSD. We measured the right and left pulmonary artery area index and also the combined pulmonary artery area index, both before and after shunt operation. The mean ± SD of these three variables before the shunt operation in the TOF group were 63.5 ± 22.5, 57.8 ± 24.9, and 121.4 ± 42.8 mm2/m2; after shunt operation they were 98.5 ± 33.6, 85.9 ± 31.9, and 184.0 ± 59.8 mm2/m2, respectively (p values <0.0001, <0.0002, and <0.0001, respectively). In the PA&VSD group the comparable values before shunt operation were 66.5 ± 16.0, 55.4 ± 10.6, and 120.9 ± 26.9 mm2/m2 and after shunt operation were 90.5 ± 22.9, 77.2 ± 24.1, and 166.6 ± 44.4 mm2/m2, respectively (p values <0.0006, <0.014, and <0.002, respectively). We also examined the effect of distensibility of pulmonary arteries by comparing the percentage change in size of the combined pulmonary artery area index in the first 4 months after shunt with those after this time (p < 0.023). There were no significant differences between left- and right-sided shunts, origin and distal pulmonary artery growth, and the TOF and PA&VSD groups.

Double orifice left atrioventricular valve—diagnosis and management of an unexpected lesion

Double orifice left atrioventricular valve is an uncommon condition. This paper describes 10 consecutive patients with the anomaly encountered over 20 months. Diagnosis was assisted by cross-sectional echocardiography and supplementary color Doppler, often requiring non-standard views and a high index of suspicion. There were three anatomical variants—those in which the orifices were equal in size (three of 10 patients), those in which they were unequal and associated with an atrioventricular septal defect (four of 10 patients), and those in which they were unequal in the absence of an atrioventricular septal defect (three of 10 patients). The unequal orifice in both anatomical situations was frequently complicated by valvar regurgitation (six of seven patients). Conservative repair was possible in three of the four patients requiring surgery to the left atrioventricular valve. This condition should be thought of more often in the differential diagnosis of left atrioventricular regurgitation in childhood.

Transesophageal echocardiographic guidance of blade atrial septostomy in children.

Transesophageal echocardiography (TEE) was used to guide blade atrial septostomy in children requiring decompression of right or left atrium. In conjunction with biplane fluoroscopy, TEE was used to position the blade septostomy catheter and to monitor the pullback from left atrium to right atrium. Subsequent balloon dilatation of atrial septum was also carried out under TEE control. The use of TEE monitoring facilitated the procedure by providing optimal views of intracardiac structures while also limiting the total radiation exposure. Cathet Cardiovasc Intervent 2001;52:200–202.

Internal mammary artery grafting in a neonate for coronary hypoperfusion after arterial switch

Coronary hypoperfusion may occur after the arterial switch operation, especially when coronary anatomy is complicated. We report successful use of the left internal mammary artery graft for a neonate with coronary hypoperfusion after the arterial switch operation for transposition of great arteries with intramural left coronary artery. Internal mammary arteriography showed a patent graft 19 months after operation.

Salvage of infected truncus repair using rifampicin-impregnated gelatin-sealed graft

An infant suffered sternal infection and ventricular septal patch dehiscence after a truncus arteriosus repair. Successful reoperation used rifampicin-impregnated gelatin-sealed Dacron to repair the recurrent ventricular septal defect and replace the contaminated conduit. This was an effective solution to a potentially lethal surgical complication.

Diagnostic Approach to Hypoplastic Right Heart and Pulmonary Atresia: Invasive and Noninvasive

Hypoplastic right heart continues to be a challenge because of its heterogenous anatomical substrate and physiological consequences. A combined echocardiographic and angiographic approach to management is presented, with the results in 39 patients (1984–1993) described. Survival has improved, and individualised surgical management has optimised medium-term outcome. Nevertheless, completed surgery still eludes at least one-third of patients.