Predictive value of intraoperative diagnosis of residual ventricular septal defects by transesophageal echocardiography.

BACKGROUND: Intraoperative transesophageal echocardiography (IOTEE) is well established as a monitoring tool during ventricular septal defect (VSD) repair to ensure complete closure of the defect. Residual shunts detected by IOTEE are common. The predictive value of IOTEE findings on the long-term course of residual shunts is not well documented, especially in regard to the need for reoperation or bacterial endocarditis prophylaxis. The objective of this study is to determine the predictive value of intraoperative IOTEE diagnosis of residual VSDs and therefore delineating the natural history of these findings. METHODS: Retrospective review of IOTEE reports of 690 consecutive patients with VSD (isolated or part of a complex lesion) was undertaken. Those were compared with transthoracic echocardiographic reports of these patients before their discharge from the hospital, and the most recent transthoracic echocardiographic examination. Positive and negative predictive values, sensitivity, and specificity of such diagnoses were then calculated from predischarge and from follow-up transthoracic echocardiographic data. RESULTS: There were 260 of 690 patients with a residual VSD on IOTEE; 24 required repeat cardiopulmonary bypass for complete closure. There were 573 patients with predischarge transthoracic echocardiographic examination; 296 had residual VSDs (125 not detected by IOTEE), and 13 defects required reoperation during the same hospitalization, 5 of which were detected by IOTEE. The positive and negative predictive values were 78% and 65%, respectively. Follow-up transthoracic echocardiographic examination of 383 local patients showed residual VSD in 57 (37 not detected by IOTEE), with positive and negative predictive values of 15% and 83%, respectively. CONCLUSIONS: Although IOTEE is sensitive enough to detect residual VSD shunts in many patients (37% of this cohort), the majority of these defects are trivial and resolve spontaneously, with a positive predictive value of only 15% on follow-up transthoracic echocardiographic examination and a rare need for reoperation.

Evaluation of myocardial ischemia after surgical repair of anomalous aortic origin of a coronary artery in a series of pediatric patients.

OBJECTIVES: We sought to prospectively evaluate evidence of myocardial ischemia after surgical repair of anomalous aortic origin of a coronary artery with an interarterial course (AAOCA). BACKGROUND: An AAOCA is a rare anomaly associated with increased myocardial ischemia and sudden death risk in children. Data evaluating ischemia after AAOCA repair are limited. METHODS: We included children who underwent AAOCA surgery between October 2001 and December 2006. They were prospectively assessed with exercise stress test (EST), stress echocardiogram (SE), and stress myocardial perfusion scan (MPS). RESULTS: Of 24 participating children, 15 (63%) were male, 16 (67%) had anomalous right coronary (ARCA), and 7 (29%) were asymptomatic. Median age was 12 (5 to 18) years; follow-up was 15 (2 to 48) months. All had unobstructed neo-coronary ostia by echocardiogram and were asymptomatic. One anomalous origin of left main coronary artery (ALCA) patient and 8 ARCA patients had post-operative evaluations suggestive of ischemia. The ALCA patient had reversible apical septal and mid-anteroseptal hypokinesis on SE. Of the ARCA patients, 2 had inferior ST-segment depression on EST; subsequently, 1 had normal tests, but the other developed anterolateral Q waves. Two patients had blunted blood pressure response with EST, 1 had fixed apical inferior hypokinesis on SE, 2 had reversible perfusion defects on MPS, and 1 had a fixed perfusion defect on MPS. CONCLUSIONS: Subclinical changes suggestive of ischemia might occur despite patent neo-coronary ostia, notably after ARCA repair. The implication of these results on indication for surgery and subsequent sudden death risk is unknown. Serial EST, SE, and MPS are essential in evaluating ongoing ischemia risk after AAOCA repair.

Atrioventricular valve morphology and dynamics in congenital heart disease as imaged with real-time 3-dimensional matrix-array echocardiography: comparison with 2-dimensional imaging and surgical findings.

OBJECTIVE: We sought to describe our initial experience with real-time 3-dimensional echocardiographic (RT3DE) imaging of atrioventricular valve (AVV) anomalies in pediatric patients to resolve a specific morphologic or dynamic detail, which, although suggested, could not be well resolved during the conventional 2-dimensional (2D)/Doppler examination. METHODS: In all, 41 patients (age range 1 day-24 years) with different AVV anomalies underwent RT3DE as part of their comprehensive echocardiographic assessment. Matrix-array transducers with a frequency range of 1 to 4 MHz were used. The first 27 patients were not consecutive, and the RT3DE findings interpreted by one examiner were compared directly with the 2D findings interpreted by a different examiner. In the following consecutive 14 patients, the RT3DE findings were compared with the surgical findings and with the interpretations of their corresponding 2D examinations by 3 examiners who were blinded to the findings of RT3DE for more objective comparison. RESULTS: RT3DE imaging was successful in resolving the raised question in all patients. The morphology of the valve leaflets and their chordal attachments, the mechanism and origin of regurgitation, and the geometry of the regurgitant volume were well delineated by RT3DE imaging. In the second group of patients, there was agreement between the morphologic details delineated by RT3DE (a total of 21 specific questions raised) and the surgical findings in those patients who had immediate surgery. On the other hand, RT3DE showed prominent AVV chordal attachments in the left ventricular outflow tract in one patient, which was not considered surgically relevant during the repair. CONCLUSIONS: Although RT3DE is still in its initial phase, has some technical limitations, and does not change the basic diagnosis made by 2D imaging, echocardiographic examination using the matrix-array transducer is a useful adjunct in delineating specific morphologic and dynamic details of the AVV in congenital heart disease.

Influence of consanguinity on the pattern of familial aggregation of congenital cardiovascular anomalies in an outpatient population: studies from the eastern province of Saudi Arabia.

BACKGROUND: Familial aggregation of congenital heart disease (CHD) has been well described in different populations, in particular those with a high consanguinity rate. Extensive genetic study of affected families has improved the understanding of basic genetics of different cardiac lesions. OBJECTIVE: To identify the role of consanguinity as a risk factor among familial cases of CHD in a stable outpatient population of a tertiary care center in the Eastern Province of Saudi Arabia. METHODS: All familial cases of CHD seen over 5 years (1996-2000) in the Division of Pediatric Cardiology were identified. The presence or absence of parental consanguinity (first cousin marriage) was defined in each of these families. RESULTS: Ninety-three cases were identified in 37 families. Twenty-three (62%) families resulted from consanguineous marriages. In 4 families where there were 2 marriages, the affected children came from the consanguineous marriage in 3 of these families. Discordant lesions occur only among non-consanguineous cases, while all consanguineous cases were concordant. Five sets of twins of the same sex (one set are monozygotic by DNA analysis) occurred among consanguineous marriages, in 3 of these both twins were affected with the same disease. Affected parents were seen in 2 families with consanguineous marriage and none in the non-consanguineous marriages. The prevalence of dilated cardiomyopathy was much higher among consanguineous cases (26 vs. 2). CONCLUSIONS: Familial aggregation of congenital heart disease is common in our population. Consanguinity is common in these families, and the distribution of congenital heart disease differs in this subgroup compared to the rest of the familial cases. Further genetic studies of these families may help to shed more light on basic genetics and the specific pathogenetic mechanisms involved.

Illustration of the additional value of real-time 3-dimensional echocardiography to conventional transthoracic and transesophageal 2-dimensional echocardiography in imaging muscular ventricular septal defects: does this have any impact on individual patient treatment?

OBJECTIVE: We sought to answer the question of whether the additional morphologic details obtained by real-time 3-dimensional (3D) echocardiographic (RT3DE) imaging of muscular ventricular septal defect (VSD) has any significant impact on treatment options of individual patient. BACKGROUND: Muscular VSD can be safely and effectively closed by interventional catheterization procedure using VSD devices under transesophageal echocardiographic (TEE) guidance. Recent application of RT3DE has shown great promise for imaging VSD with better display of the exact geometry, size, and location of the defect. METHODS: Nineteen patients with different types of VSDs were imaged with RT3DE matrix-array transducer; there were 6 cases with muscular VSD. Based on standard transthoracic echocardiographic and TEE imaging, one patient was considered a good candidate for perventricular VSD device occlusion, three patients were considered for surgical closure, and in two patients no intervention was deemed necessary. RESULTS: RT3DE successfully displayed the exact morphology of the VSD in all 6 patients, whereas transthoracic echocardiography and TEE showed the defect as a dropout with variable diameter in different views. Such planer images did not accurately predict the exact morphology in the patient in whom device occlusion was considered and the device embolized to the left ventricle in a few heartbeats. Surgical circular patch was used in two patients and primary suture was used in two patients in agreement with the 3D morphology. In two patients the 3D morphology of the VSD was small enough that no intervention was considered. CONCLUSIONS: RT3DE imaging of muscular VSD can accurately display the exact geometry of the defect, which can have significant impact on treatment strategies of individual patients. This new imaging modality should be an important adjunct to the standard transthoracic echocardiographic and TEE imaging of these defect before any intervention.

Real-time 3-dimensional echocardiographic imaging of congenital heart disease using matrix-array technology: freehand real-time scanning adds instant morphologic details not well delineated by conventional 2-dimensional imaging.

OBJECTIVE: We sought to investigate whether real-time (RT) 3-dimensional echocardiography (RT3D) using matrix-array technology could resolve a specific morphologic detail that could not be well resolved during conventional 2-dimensional (2D)/Doppler echocardiographic imaging of congenital heart disease. BACKGROUND: Although 2D echocardiography is currently the primary imaging modality of congenital heart disease, there are still some anatomic details that cannot be well delineated by that modality. METHODS: In all, 70 patients underwent RT3D examination using matrix-array transducer. Applying the standard sweeping technique as for 2D imaging, freehand RT imaging was used for immediate feedback. The 2D examinations of the last 23 consecutive patients were used to validate the additional value of RT3D by 3 examiners blinded to the findings of RT3D. RESULTS: The adequacy of 2D imaging to resolve the morphologic detail in question ranged from 30% to 70%, whereas RT3D imaging was considered successful in delineating the morphologic detail in 80% to 100% of patients instantly. The image resolution was superior or equivalent to that obtained by 2D imaging. Heart valves, septal defects, and volumetric valvular and vascular color flow morphologies were well delineated by RT3D. CONCLUSION: Matrix-array RT3D is a significant breakthrough technology that allowed instant visualization of cardiac anatomic details that could not be well delineated by 2D imaging. Further improvements of some technical limitations should make RT3D matrix-array cardiac imaging a significant modality in the field of echocardiographic imaging of congenital cardiac anomalies.

Spontaneous Obstruction of Interatrial Communication in Infants with Atrioventricular Valve Atresia or Stenosis in the Neonatal Period: Serial Echocardiographic-Doppler Study.

BACKGROUND AND METHODS: The objective of this serial echocardiographic study was to document the critical size of the interatrial communication in infants with atrioventricular valve atresia, with which spontaneous obstruction is most likely to occur. Spontaneous obstruction of interatrial communication (patent foramen ovale) has been reported to occur in infants with atrioventricular valve atresia or severe stenosis. Atrial septostomy or septectomy therefore may be considered during initial palliation of these infants. Serial two-dimensional Doppler echocardiographic examinations were performed on 11 newborn infants with complex cyanotic congenital cardiac lesions for whom Fontan procedure was contemplated. These newborn infants had either mitral or tricuspid valve atresia or severe stenosis other than hypoplastic left heart syndrome. The size of the foramen ovale was measured from the subcostal frontal, long-axis oblique, and parasagittal views. Conventional Doppler and color flow mapping of the interatrial shunt also was obtained on each patient. RESULTS: The size of the foramen ovale was 5 mm in nine patients at birth. Five of these patients underwent Blalock-Taussig shunt in the neonatal period for severely restricted pulmonary blood flow. In three patients, surgery was postponed due to severe systemic (noncardiac) disease. One patient underwent the hemi-Fontan procedure without a previous shunt. In all nine patients, the foramen ovale remained the same size or became progressively smaller beyond the neonatal period, and atrial septectomy was performed in five patients after initial palliation. In the remaining two patients (who underwent Blalock-Taussig shunt as initial palliation), the foramen ovale measured >/=10 mm at birth and became progressively larger on subsequent follow-up studies. Atrial septectomy was not necessary in these patients. In the first nine patients, Doppler technique did not detect any obstructive pattern during the first 2-4 weeks of life, after which it became progressively obstructive (i.e., >2 m/sec and nonphasic). CONCLUSIONS: In newborn infants with either atrioventricular valve atresia or severe stenosis, the interatrial communication, which may not appear restrictive by Doppler at birth, becomes progressively obstructed beyond the neonatal period. If the size of the communication is <10 mm in diameter, as measured by two-dimensional echocardiography, atrial septectomy should be performed as an integral part of any initial palliation to protect the pulmonary vascular bed for subsequent successful Fontan repair.