An uncommon echocardiographic marker for anomalous origin of the left coronary artery from the pulmonary artery: visualization of intercoronary collaterals within the ventricular septum.

A rare and striking echocardiographic finding with color-flow Doppler--visualization of intercoronary collaterals within the ventricular septum--in an asymptomatic 5-year-old girl is reported. It strongly suggests anomalous origin of the left coronary artery from the pulmonary artery. Several additional echocardiographic features can secure the diagnosis and allow one to proceed with surgical correction without confirmatory cardiac catheterization and angiocardiography. The ages of our patient and of the seven cases reported previously suggest that these collaterals will more likely be identified beyond infancy and in relatively asymptomatic patients with this congenital anomaly.

Electrocardiographic and echocardiographic features that distinguish anomalous origin of the left coronary artery from pulmonary artery from idiopathic dilated cardiomyopathy.

Many authors have reported noninvasive means of diagnosing anomalous left coronary artery from pulmonary artery (ALCAPA) and differentiating ALCAPA from idiopathic dilated cardiomyopathy (DCM). Systematic evaluation using these noninvasive diagnostic modalities is not available. To distinguish between ALCAPA and DCM using a systematic approach, we examined 23 patients with ALCAPA (age 1 month to 23 years, median 7 months) and 23 patients with DCM (age 5 days to 16 years, median 6.6 months). Standard 12-lead electrocardiograms (ECG) and 2-dimensional (2-D) and color Doppler echocardiograms were performed. A logistic regression model was applied using ALCAPA diagnosis as the dependent variable and ECG and echocardiographic findings as independent variables. A scoring system was created to establish the ALCAPA diagnosis based on results from the logistic regression. On the logistic regression, the ECG feature of QT pattern in aVL (Q wave > or = 3 mm deep with an inverted T wave) and echocardiographic features of right coronary artery diameter to aortic annulus ratio > or = 0.14, increased papillary muscle echogenicity, and Doppler color flow of LCA from aorta or pulmonary artery were the most significant differentiating features between the ALCAPA and DCM groups. A scoring system was developed using the previous five variables and assigning a score of 1 to each variable (-1 to Doppler color flow of LCA from aorta). The scoring system had sensitivity of 100% and specificity of 91% for ALCAPA diagnosis. Compared with previous reported diagnostic features in differentiating ALCAPA and DCM, the scoring system had a much higher specificity and positive predictive value. In conclusion, we selected the most useful ECG and echocardiographic features to differentiate between ALCAPA and DCM and created a scoring system to aid clinical diagnosis. This scoring system may be useful in evaluating children with acute congestive heart failure.

Staged surgical approach to neonates with aortic obstruction and single-ventricle physiology.

BACKGROUND: The surgical management of neonatal systemic outflow obstruction and complex single ventricle pathology is variable. METHODS: In 15 neonates (12 boys and 3 girls) with complex forms of single-ventricle pathology and aortic coarctation or interruption, an initial strategy of banding the pulmonary artery and repair of the obstruction from a left thoracotomy was undertaken. RESULTS: The median age at operation was 6 days (range 2 to 33 days) and the median weight was 3.3 kg (range 2 to 4.6 kg). There were no early deaths and one late death after the initial surgical palliation. Of the 14 survivors, 8 have undergone a bidirectional cavopulmonary anastomosis. The median age for bidirectional Glenn was 9.75 months (range 3.5 to 26 months). Seven infants have required Damus-Kaye-Stansel reconstruction for subaortic obstruction (one early death). The median age of the Damus-Kaye-Stansel procedure was 4 months (range 3 weeks to 9 months). Thirteen of 15 patients (87%) are alive and 6 have proceeded to a Fontan operation (median follow-up 68 months). A single failing Fontan required takedown to bidirectional Glenn and central shunt. CONCLUSIONS: Our experience suggests that this high-risk subgroup of neonates with aortic obstruction and single-ventricle pathophysiology is safely managed by initial pulmonary artery banding palliation and repair of aortic obstruction. This strategy, careful surveillance, and early relief of subaortic stenosis can maintain acceptable anatomy and hemodynamics for later bidirectional Glenn and Fontan procedures.

The incisional pulmonary artery band.

Occasionally early definitive repair of congenital heart disease carries prohibitive mortality, and interval pulmonary artery banding is necessary to protect the pulmonary arterial bed and improve systemic perfusion or prepare a systemic left ventricle for a later arterial switch operation. We describe our technique for effectively banding the pulmonary artery.

Derivation of input function from FDG-PET studies in small hearts.

UNLABELLED: The extraction of pure arterial time-activity curves (TACs) from dynamic PET images of a small animal heart using factor analysis of dynamic structures (FADS) was found to be unsuccessful due to the small size of the cardiac chamber that causes extensive mixture of TACs of different structures. METHODS: In this study, we used digital phantoms of the left ventricle (LV cavity size: 1-2 cm) and small monkey (LV cavity size: approximately 2 cm) dynamic FDG PET studies to evaluate FADS for extracting the pure blood-pool TACs by adding a single blood sample (taken at a late scan time) constraint. RESULTS: In the digital phantom studies, spillover fractions in the extracted blood-pool TACs using FADS without a blood sample constraint (FADS(-)) and with a blood sample constraint (FADS(+)) were 3%-91% and < 3%, respectively. In the monkey studies (n = 4), FADS(+) extracted blood-pool TACs matched well with the arterialized well counter measurements (% differences of curve integration; FADS(-) < 82%; FADS(+) < 9%). The microparameters (K1*, k2*, k3*, k4*) and macroparameters (Knlr), obtained from the FADS(+) blood-pool TACs, were similar to those obtained from plasma samples in a three-compartment model fitting (% differences of Knlr:phantom studies < 5%; monkey studies < 9%). CONCLUSION: The FADS technique with a single-blood sample has the potential to extract the pure blood-pool TACs directly from dynamic PET images of a small animal without multiple blood sampling, region of interest definition or spillover correction.

Developmental changes in brain metabolism in sedated rhesus macaques and vervet monkeys revealed by positron emission tomography.

The present positron emission tomography study used 2-deoxy-2[18F]fluoro-D-glucose to examine age-related changes in local cerebral metabolic rates for glucose (LCMRglc) in sedated rhesus macaques and vervet monkeys. Nineteen vervet monkeys were scanned in a cross-sectional design, which consisted of three age groups (birth to 59 d, 60-179 d, 180 d to adult) that captured the developmental period of greatest synaptic density within the second group. Two rhesus and two vervet monkeys were also examined longitudinally. Subjects were sedated throughout the procedure with a combination of ketamine and midazolam. Longitudinal and cross-sectional analyses of 20 brain regions indicated lowest LCMRglc in rhesus and vervet monkeys < 2 months of age (about 60% of adult levels). These metabolic rates more than doubled to approximately 155% of adult levels after the second postnatal month, coincident with transient synaptic overproduction and increased sociobehavioral activity. LCMRglc remained high until 6 months postnatally, coincident with continued dendritic growth and the emerging sociobehavioral independence of the young monkey. After 6 months, LCMRglc decreased gradually to adult levels. Thus, the period between 2 and 6 months is a time of peak metabolic activity, which coincides with increased histologic and behavioral activity in the developing monkey.

Aortic implantation of anomalous left coronary artery. An improved surgical approach.

Anomalous origin of the left coronary artery from the pulmonary artery may present a technical challenge. Direct implantation of the anomalous left coronary artery into the aorta to provide a two coronary artery system is the preferred surgical approach. We describe a modification of this technique to allow anastomosis of the anomalous left coronary artery with the excised button of pulmonary artery from within the lumen of the aorta. We have used this procedure in six children and one adult with anomalous left coronary artery with favorable outcome. The potential benefits of this modified technique are (1) improved operative exposure, (2) ability to implant the anomalous left coronary artery in the appropriate sinus, (3) avoidance of aortic valve damage or distortion because of improved exposure, and (4) applicability to patients of all ages.

Aortic to right ventricular shunt for pulmonary atresia and intact ventricular septum.

Acute or chronic myocardial ischemia may develop in patients with pulmonary atresia with intact ventricular septum and right ventricular-dependent coronary circulation. In such cases an aorta to right ventricle shunt may be used to reverse this ischemia. This report summarizes our experience with the placement of an aortic to right ventricular shunt in 5 patients. The shunts were made of Gore-Tex and ranged from 4 mm to 8 mm. Associated procedures were bidirectional Glenn (n = 2) and Fontan (n = 2). All 5 patients survived the procedure with documented early graft patency and no evidence of ischemia.

Selective anterograde coronary arteriography in neonates with d-transposition of the great arteries: accuracy and safety.

OBJECTIVES: This study was designed to evaluate the accuracy and safety of selective anterograde coronary arteriography for the identification of the origin and branching pattern of the three main coronary arteries in neonates with d-transposition of the great arteries. BACKGROUND: Definition of coronary artery anatomy is important in neonates with d-transposition of the great arteries who are considered for the arterial switch operation. Balloon occlusion aortography defines coronary artery anatomy in most but not all cases. We have described a technique for selective anterograde coronary arteriography. METHODS: Between March 1987 and May 1991, 17 neonates underwent selective anterograde coronary arteriography and 29 patients had balloon occlusion aortography. After venous access was gained, a mesenteric catheter was used to engage the coronary ostia for contrast injection. All angiograms were reviewed by three independent observers and the coronary artery diagnoses were compared with operative findings. Complications with the catheterization procedure were also recorded. RESULTS: The accuracy of defining coronary artery anatomy with selective anterograde coronary arteriography (98 +/- 2%) was significantly (p < 0.05) greater than that achieved with balloon occlusion aortography (69 +/- 6%). There were no deaths with catheterization in either study group. Morbidity was similar in the groups with balloon occlusion aortography (7%) and selective anterograde coronary arteriography (6%) (p > 0.05) and was related to transient bradycardia induced by catheter manipulation in the right ventricle. No patient in either study group experienced cardiac ischemia. CONCLUSIONS: Selective anterograde coronary arteriography is an accurate and safe technique for the definition of coronary artery pattern in neonates with d-transposition of the great arteries.