Diastolic biomechanics in normal infants utilizing MRI tissue tagging.

BACKGROUND: Most of what is known about diastolic function in normal infants is derived from flow and pressure measurements. Little is known about regional diastolic strain and wall motion. METHODS AND RESULTS: Magnetic resonance tissue tagging was performed in 11 normal infants to determine regional diastolic strain and wall motion. Tracking diastolic motion of the intersection points and finite strain analysis yielded regional rotation, radial displacement, and E(1) and E(2) strains at 3 short-axis levels (significance was defined as P<0.05). E(2) "circumferential lengthening" strains were significantly greater at the lateral wall, regardless of short-axis level, whereas E(1) "radial thinning" strains were similar in all wall regions at all short-axis levels. In general, no differences were noted in strain dispersion within a wall region or in endocardial/epicardial strain at all short-axis levels. At all short-axis levels, septal radial motion was significantly less than in other wall regions. No significant differences in radial wall motion between short-axis levels were noted. Rotation was significantly greater at the apical short-axis level in all wall regions than in other short-axis levels, and it was clockwise. At the atrioventricular valve, septal and anterior walls rotated slightly clockwise, whereas the lateral and inferior walls rotated counterclockwise. CONCLUSIONS: Diastolic biomechanics in infants are not homogeneous. The lateral walls are affected most by strain, and the septal walls undergo the least radial wall motion. Apical walls undergo the most rotation. These normal data may help in the understanding of diastolic dysfunction in infants with congenital heart disease.

Fetal hydrops in a newborn with hypoplastic left heart syndrome: tricuspid valve "stopper".

Fetal hydrops in a newborn infant with hypoplastic left heart syndrome led to the discovery of tricuspid stenosis and insufficiency from an unusual malformation of the right venous valve of the embryonic sinus venosus. This unfortunate combination of lesions precluded surgical palliation for the hypoplastic left heart.

Subcostal two-dimensional echocardiographic identification of anomalous attachment of septum primum in patients with left atrioventricular valve underdevelopment.

Five variations of atrial septal morphology occur in hypoplastic left heart syndrome. One variety, termed anomalous attachment of septum primum, has been described only in necropsy series. Two-dimensional echocardiography was utilized to determine the incidence of this anomaly in patients with left atrioventricular (AV) valve underdevelopment, including those with other ventriculoarterial alignments, such as transposition of the great arteries. Forty-eight (37%) of 129 patients with normally aligned great arteries (and two ventricles) had anomalous attachment of septum primum. Ten (34%) of 29 patients with double outlet right ventricle and left AV valve underdevelopment had this anomaly. Four (50%) of eight patients with single ventricle exhibited this atrial septal variant. The most reliable view to identify anomalous attachment was the subcostal left oblique-equivalent cut. Recognition of atrial septal morphology has implications for preoperative and intraoperative management of patients with left AV valve underdevelopment. The similar prevalence of this atrial septal variant in patients with normally aligned great arteries, double outlet right ventricle and transposed great arteries suggests that there may be a common mechanism for left AV valve underdevelopment that is independent of the development of the arterial portion of the heart.

Morphometric analysis of unbalanced common atrioventricular canal using two-dimensional echocardiography.

OBJECTIVES: This study was designed to define morphometric echocardiographic variables of unbalanced common atrioventricular canal (CAVC) that could aid in appropriate referral for surgical repair. BACKGROUND: Unbalanced CAVC has a high surgical mortality rate. This may be secondary to inappropriate referral of some patients for two-ventricle repair (closure of septal defects) instead of single-ventricle repair (Norwood palliation and Fontan operation). METHODS: The echocardiograms of 103 patients with CAVC were retrospectively reviewed. In the subcostal left anterior oblique view, the area of the atrioventricular (AV) valve aportioned over each ventricle was measured, and an AV valve index (AVVI) was calculated as left/right valve area. The ventricular cavity ratio between the two ventricles was estimated as left ventricular length times width divided by right ventricular length times width. These variables were correlated with surgical referral and outcome. RESULTS: Patients previously categorized as having balanced CAVC all had AVVI > 0.67 (n = 77). Of the patients with unbalanced CAVC (n = 26), 11 had ductal-dependent circulation and underwent Norwood palliation (AVVI 0.21 +/- 0.13, mean +/- SD), and 15 had two-ventricle repair (AVVI 0.51 +/- 0.12, p < 0.0001). Of these 15 patients, 9 have survived, with no difference in mean AVVI between survivors and nonsurvivors (0.52 +/- 0.11 versus 0.49 +/- 0.13, p = 0.72). For all 103 patients, AVVI correlated with ventricular cavity ratio. However, of the unbalanced CAVC group who underwent two-ventricle repair, three nonsurvivors had a discrepancy between AVVI and ventricular cavity ratio (low AVVI but normal ventricular size). A large ventricular septal defect was present in all six nonsurvivors but in only four of nine survivors (p < 0.05). CONCLUSIONS: Echocardiographic morphometry is useful in defining unbalance in CAVC. If AVVI is < 0.67 in the presence of a large ventricular septal defect, a single-ventricle approach to repair should be considered.

Growth characteristics of the aortic arch after the Norwood operation.

OBJECTIVES: We sought to characterize the growth of the reconstructed aortic arch after the Norwood operation (NO). BACKGROUND: The first stage of surgical palliation of hypoplastic left heart syndrome (HLHS), the NO, includes augmentation of the aortic arch with homograft. Growth characteristics of the reconstructed aortic arch, which is comprised of both native aortic tissue and homograft, have not been characterized. METHODS: Retrospectively, we examined the serial echocardiograms of 50 patients with HLHS who underwent NO to determine the diameter of the reconstructed transverse arch. Measurements were taken immediately after NO and at two other points (1 to 11 years of age). In addition, the autopsy specimens of 10 other patients with HLHS who underwent NO were examined to determine the contribution of native aortic tissue to the overall size of the reconstructed arch at the time of death (12 to 34 months). RESULTS: The diameter of the transverse aorta increased after NO in all subjects. Its rate of growth paralleled that seen in the normal population, though the reconstructed arch had a significantly larger diameter throughout childhood. Examination of autopsy specimens demonstrated a mean increase in circumference of the native aortic tissue of 0.67 cm (p value <0.01), whereas there was no significant change in homograft circumference. CONCLUSIONS: After reconstruction of the aortic arch in HLHS, the diameter of the arch continues to increase throughout childhood, and this increase is due to growth of the native aortic tissue.

Origin of the left coronary artery from the right pulmonary artery.

Origin of the left coronary artery from the right pulmonary artery has rarely been documented. This is the first such case in a heart with an intact ventricular septum and paraductal coarctation of the aorta. Although an antemortem diagnosis was made and the anomalous left coronary artery was ligated, the patient, a 3 1/2 month old infant, died 1 day after surgery. Autopsy confirmed the diagnosis, but revealed that the left coronary artery was dominant. It is believed that the fatal outcome in the infant was, in part, due to the dominance of the left coronary artery and the effects of the coarctation on the already ischemic left ventricle.

Coronary artery abnormalities and right ventricular histology in hypoplastic left heart syndrome.

OBJECTIVES: To determine whether right ventricular preservation is compromised in any anatomic subgroup of hypoplastic left heart syndrome, we assessed the coronary artery anatomy and myocardial histologic features of 151 postmortem specimens with hypoplastic left heart syndrome. BACKGROUND: Although previous studies have shown that coronary artery abnormalities are more prominent in the subgroup of patients who have a patent mitral valve and obstructed aortic valve, it has not been established that these abnormalities compromise right ventricular perfusion. METHODS: Eighty-nine specimens with a patent mitral valve and aortic atresia, 52 with mitral and aortic atresia and 10 with patent mitral and aortic valves were examined. Histologic sections of the right ventricle, left ventricle and coronary arteries were obtained from 64 study hearts and 5 control hearts. RESULTS: Gross coronary anomalies included coronary-cameral communications (n = 29), single left coronary artery (n = 2), single right coronary artery (n = 1) and tortuosity (n = 19). Coronary-cameral communications and tortuosity were significantly associated with the subgroup that had mitral hypoplasia and aortic atresia. Coronary artery wall thickness relative to lumen diameter was not different among the subgroups. No areas of coronary artery stenosis or interruption were discovered. Although endocardial fibroelastosis of the left ventricle was strongly associated with the mitral hypoplasia and aortic atresia group, the right ventricular histologic findings were similar in all subgroups and were not differentially affected by older age. CONCLUSIONS: Although the incidence of coronary abnormalities is greater in patients with mitral hypoplasia and aortic atresia, in this study there was no apparent difference in perfusion of the right ventricle among the anatomic subgroups of hearts with hypoplastic left heart syndrome.

Late ventricular geometry and performance changes of functional single ventricle throughout staged Fontan reconstruction assessed by magnetic resonance imaging.

OBJECTIVES: We sought to test the hypothesis that late ventricular geometry and performance changes occur in functional single ventricles as they progress through staged Fontan reconstruction. BACKGROUND: Indexes of ventricular geometry and performance are important in evaluating the functional state of the heart. Magnetic resonance imaging determines these indexes in complex ventricular shapes with minimal geometric assumptions. Previous studies have shown that 1 week after hemiFontan, the mass/volume ratio markedly increases. METHODS: Multiphase, multislice, spin echo (n = 5) and cine (n = 30) magnetic resonance imaging was performed in 35 patients with a functional single ventricle (1 week to 12 years old) at various stages of Fontan reconstruction (15 in the pre hemiFontan stage, 11 after [6 to 9 months] the hemiFontan procedure and 9 after [1 to 2 years] the Fontan procedure). Volume and mass were calculated at end-systole and end-diastole. Ventricular output was then obtained. Ventricular centroid motion was also calculated. RESULTS: No difference was noted (power > 72%) from the pre hemiFontan stage to 6 to 9 months after the hemiFontan procedure in (mean +/- SD) end-diastolic volume (104 +/- 24 vs. 123 +/- 40 cc/m2), mass (171 +/- 46 vs. 202 +/- 61 g/m2), ventricular output (7.9 +/- 2.2 vs. 6.6 +/- 2.4 liters/min per m2) or centroid motion (6.9 +/- 2.8 vs. 6.7 +/- 2. mm/m2). Patients in the Fontan group demonstrated a marked decrease in all indexes, indicating significant volume unloading and decrease in mass and ventricular performance. Mass/volume ratio was not significantly different among all three groups. CONCLUSIONS: No geometric and performance changes from the volume-loaded stage are noted 6 to 9 months after the hemiFontan procedure; however, major changes occur 1 to 2 years after the Fontan procedure. The dramatic changes in the mass/volume ratio seen early after the hemiFontan procedure were not detected at 6 to 9 months. Furthermore diminution of mass, volume and ventricular performance are present at least 2 years after the Fontan procedure.

Association of chromosome 22q11 deletion with isolated anomalies of aortic arch laterality and branching.

OBJECTIVES: The purpose of this study was to determine the frequency of chromosome 22q11 deletions in patients with isolated anomalies of the aortic arch and its branches. BACKGROUND: Chromosome 22q11 deletions are often present in patients with certain forms of congenital cardiovascular disease, including tetralogy of Fallot, truncus arteriosus and interruption of the aortic arch. Among patients with these anomalies, chromosome 22q11 deletion is more common in those with abnormal aortic arch laterality or branching. METHODS: We studied 66 patients with isolated anomalies of the aortic arch and no associated intracardiac defects for deletions within chromosome 22q11, using fluorescence in situ hybridization with the cosmid probe N25 (D22S75). Arch anomalies included: double aortic arch (n = 22); right aortic arch with aberrant left subclavian artery (n = 28); right aortic arch with mirror-image branching and a vascular ring formed by a left-sided ductus from the descending aorta (n = 5); right aortic arch with mirror-image branching and no vascular ring (n = 4); and left aortic arch with aberrant right subclavian artery (n = 7). In addition, four patients had a cervical aortic arch, four had aortic coarctation and six had hypoplasia/atresia of the proximal pulmonary arteries. RESULTS: Chromosome 22q11 deletions were found in 16 patients (24%) across the full spectrum of anomalies studied. Among the morphologic variables analyzed, only hypoplasia/atresia of the proximal pulmonary arteries correlated with the deletion (p = 0.03). Among patients with a double arch, the frequency of chromosome 22q11 deletion was higher in those with an atretic minor arch than it was in those with a patent minor arch (p = 0.02). CONCLUSIONS: Chromosome 22q11 deletion is associated with isolated anomalies of laterality or branching of the aortic arch in 24% of cases in our series. These findings should alert the clinician to consider deletion screening in patients with isolated anomalies of the aortic arch.

A simplified approach for assessment of intracardiac baffles and extracardiac conduits in congenital heart surgery with two- and three-dimensional magnetic resonance imaging.

BACKGROUND: Intracardiac baffles and extracardiac conduits have been used in the reconstructive surgery of a broad spectrum of congenital cardiac malformations. Periodic evaluation of these structures may not lend itself readily to echocardiographic and angiographic imaging. The purpose of the study was to describe the experience of our institution with the use of magnetic resonance imaging (MRI) in evaluating conduits and baffles and to describe the simplified approach we developed to image these structures, which allows for grouping individual lesions into broad categories. METHODS AND RESULTS: We retrospectively reviewed our MRI experience in visualizing these structures from 1989-1996. One hundred thirty-nine patients underwent MRI to visualize 144 structures (116 baffles, 28 conduits). The 116 baffles included 86 Fontan, 16 Mustard, 6 Senning, 6 left ventricle to aorta, 1 right ventricle to aorta, and 1 pulmonary vein to left atrium baffle. The 28 conduits included 15 right ventricle to pulmonary artery, 4 left ventricular apical to aorta, 2 left ventricle to pulmonary artery, 3 aorta to aorta, 2 inferior vena cava to left atrium conduits, and 2 aortic root replacements. Of the 3 aortic-aortic conduits, 1 was in conjoined twins. Both inferior vena cava-left atrial conduits were in a Baffes procedure. An infectious mass missed by echocardiography in a right ventricle to pulmonary artery conduit was visualized by MRI. With multiplanar reconstruction, contiguous images were stacked atop each other and resliced to define the salient points of the anatomy. Three-dimensional reconstruction further added to this delineation. All structures were visualized successfully, and an assessment of obstruction was made. Multiple examples of conduit and baffle narrowing were diagnosed by spin echo and cine MRI and were subsequently confirmed by catheterization and surgical inspection. CONCLUSION: MRI, with multiplanar and 3-dimensional reconstruction, is useful in examining the variety of baffles and conduits used in congenital heart surgery. MRI can add to the care of patients whose echocardiographic windows or whose angiographically overlapping structures do not allow adequate delineation of conduits and baffles.

Acquired neuropathologic lesions associated with the hypoplastic left heart syndrome.

This study details the type, frequency, clinical presentation, and etiologic associations of acquired brain lesions in 40 infants with the hypoplastic left heart syndrome encountered during a 52-month interval. Detailed postmortem neuropathologic examinations showed that 55% of the infants were free of acquired brain lesions. However, the other 45% had combinations of hypoxic-ischemic lesions and intracranial hemorrhage. Central nervous system perfusion and glucose-oxygen delivery appeared to be important factors in the occurrence of hypoxic-ischemic lesions or intracranial hemorrhage, whereas acidosis and hypercarbia were not. Cerebral necrosis may be a predisposing factor for a major intracranial hemorrhage. A duration of cardiopulmonary bypass with hypothermic total circulatory arrest longer than 40 minutes was associated with a higher incidence of acquired neuropathology. These results indicate that the majority of infants with hypoplastic left heart syndrome are free of acquired neuropathology and suggest practical ways to reduce the risks in the others.

Congenital brain anomalies associated with the hypoplastic left heart syndrome.

This study details the type, frequency, and clinical presentation of developmental brain anomalies in 41 infants with the hypoplastic left heart syndrome encountered during a 52-month interval. Overall, 29% of the infants had either a major or minor central nervous system abnormality. Overt central nervous system malformations, including 3 cases of agenesis of the corpus callosum and 1 case of holoprosencephaly, were seen in 4 infants (10%). Micrencephaly (brain weight at autopsy more than 2 SDs below the mean for age) was found in 27% of the infants. An immature cortical mantle was seen in 21% of the study group. Seven infants (17%) had specific recognizable patterns of malformation. The absence of dysmorphic physical features did not preclude overt or subtle central nervous system malformations. Conversely, the presence of dysmorphic features did not reliably indicate an underlying brain anomaly. Infants who had hypoplastic left heart syndrome as one of multiple nonneurologic malformations were more likely to have micrencephaly than those infants with hypoplastic left heart syndrome as an isolated abnormality. Occurrence of developmental neuropathology was elevated in those infants with hypoplastic left heart syndrome who did not have a recognizable pattern of malformation but who were small for gestational age, microcephalic, or had ocular abnormalities. Infants with hypoplastic left heart syndrome deserve careful genetic, opthalmologic, and neurologic evaluations, imaging of their intracranial anatomy, and long-term neurologic follow-up.

Can echocardiography predict the presence or absence of endocardial fibroelastosis in infants <1 year of age with left ventricular outflow obstruction?

This study assesses the use of echocardiography in detecting endocardial fibroelastosis in 32 infants with obstructive left-sided lesions. The data demonstrate that neither endocardial echo-brightness nor geometric variables of the left ventricle allow for accurate prediction of the presence of endocardial fibroelastosis.

Angiographic, echocardiographic, and three-dimensional magnetic resonance imaging of extracardiac conduits in congenital heart disease.

Visualization of extracardiac conduits was evaluated comparing angiography, echocardiography, and magnetic resonance imaging with 3-dimensional reconstructions. Magnetic resonance imaging afforded visualization of the conduit in its entirety in a higher percentage of patients than the other 2 imaging modalities.

Postmortem echocardiography and tomographic anatomy of hypoplastic left heart syndrome after palliative surgery.

A study was undertaken to improve the understanding of the 3-dimensional (3-D) topology of a complex surgical reconstruction. The pathologic anatomy was investigated by first fixing postmortem heart specimens in such a way as to preserve the 3-D relations. Next, a technique for postmortem 2-D echocardiography was developed to aid in selection of tomographic planes for sectioning the specimens. Subsequent adjustment of planes of section was made to better show particular facets of the anatomy. The material for this investigation was drawn from cases of hypoplastic left heart syndrome after surgical palliation by the Norwood procedure. Three potential hemodynamic sequelae--restrictive inter atrial communication, aortic obstruction at any level and distortion of the pulmonary artery confluence--served as the anatomic focus for this study. Careful preservation of 3-D topology and postmortem 2-D echocardiographic imaging coupled with tomographic sectioning of specimens led to development of new, clinically relevant echocardiographic views for imaging specific atrial septal and aortic arch anomalies. These techniques offer insight into the spectrum of anatomic sequelae of this type of surgery and may be applied to echocardiographic imaging of patients and refinement of surgical technique for other forms of complex congenital heart disease.

Magnetic resonance imaging of constant total heart volume and center of mass in patients with functional single ventricle before and after staged Fontan procedure.

It has been previously demonstrated that total heart volume (contents of pericardium) throughout the cardiac cycle varies by < 5% and location of center of mass by < 3 mm. The hypothesis has thus developed that for maximal efficiency, the heart should expend minimal energy in displacing extracardiac structures by maintaining a constant intracycle total heart volume and center of mass. This is achieved in the normal heart mainly by a piston-like movement of the atrioventricular valve plane toward the ventricular apex. As this has never been studied in the single ventricle heart or at various stages of Fontan reconstruction, it is conceivable that these patients may not exhibit the constancy of total heart volume and location of center of mass, which may lead to a poor outcome in some. The total heart volume and center of mass relationship was therefore evaluated in 25 children (0.4 to 237 months) with functional single ventricles at all stages of Fontan reconstruction using multiphase, multislice spin-echo or cine-magnetic resonance imaging. No significant difference was seen in variation between total heart volume and maximal volume between patients before bilateral cavopulmonary anastomosis (hemiFontan) (5.1 +/- 2.9%), after hemiFontan (3.6 +/- 1.6%) and after Fontan (8.2 +/- 7.7%); however, in 4 of 10, 1 of 8 and 5 of 7 patients, respectively, the total heart volume varied by < 5%. Across surgical subgroups, significant differences were found in the center of mass displacement (total, anteroposterior and superoinferior planes) but not in directionality of displacement.(ABSTRACT TRUNCATED AT 250 WORDS)

Effect of persistent left superior vena cava on hemodynamic calculations.

When comparing means, there is no difference between right and left SVC saturations, and the difference does not affect cardiac output calculations. Because of the wide variability between right and left SVC saturations and their calculated cardiac output within patients, along with the higher correlation of left versus right SVC saturation with pulmonary artery saturation, the Fick-derived cardiac index calculation should be interpreted with caution when a left SVC is present. A prospective study with near-simultaneous sampling in all vessels should be undertaken.

Double outlet right ventricle (S,D,L) with subaortic ventricular septal defect and pulmonary stenosis. Report of six cases.

The clinical, hemodynamic, angiocardiographic and pathologic findings are presented in an infrequent but surgically correctable type of double outlet right ventricle. This study is based on six cases, one with autopsy confirmation. In all, the viscera and atria were in situs solitus (S). A ventricular d-loop was present (D). There was I-malposition of the great arteries, the aorta being to the left of, and anterior to, the pulmonary artery (L). Hence, this anomaly may conveniently be represented as double outlet right ventricle (S,D,L,). The ventricular septal defect to the ventricular septum. A bilateral conus was present beneath both the aortic and pulmonary valves, preventing any semilunar-atrioventricular fibrous continuity. The subpulmonary conus was poorly expanded, resulting in pulmonary infundibular and valvular (annular) stenosis. The clinical features were those of cyanosis, clubbing and accentuation of the second heart sound in the pulmonary area (related to aortic valve closure). There was a systolic ejection murmur along the upper left sternal border, related to pulmonary outflow tract stenosis. Selective right and left ventricular angiocardiography was diagnostic. Relatively early surgical correction is suggested to minimize the progression of pulmonary infundibular stenosis and to avoid acquired atresia. In this malformation, pulmonary outflow tract reconstruction is more difficult than in tetralogy of Fallot because of the rather posterior location of the pulmonary outflow tract, and because the right coronary artery crosses the stenotic pulmonary outflow tract in front of the pulmonary valve.

Massive hemoptysis secondary to pulmonary arteriovenous fistula. Treatment by a catheterization procedure.

Massive pulmonary hemorrhage secondary to an acquired arteriovenous fistula is a rare event associated with high mortality. Cotton wads mounted on steel coils were inserted by percutaneous catheter and successfully occluded a pulmonary arteriovenous fistula in a patient who had massive hemoptysis and contraindications to thoracotomy.

Transposition of the great arteries with aortic arch obstruction. Anatomical review and report of surgical management.

Transposition of the great arteries (S,D,D) is a common congenital cardiovascular malformation that is occasionally associated with ventricular septal defect and left ventricular outflow tract obstruction. Recently right ventricular outflow tract obstruction associated with an aortic arch anomaly has been recognized as an infrequent but important variant of transposition of the great arteries, and this constellation presents a unique surgical challenge. Five infants with this constellation whose systemic circulation was dependent on flow through the ductus arteriosus have undergone definitive surgical treatment with four survivors. An anatomical review of 129 specimens with transposition of the great arteries revealed that 17% had right ventricular outflow tract obstruction and 7% had associated aortic arch obstruction as well. All specimens with aortic arch obstruction and ventricular septal defect except one had a malalignment type ventricular septal defect. Although several treatment options may be considered, recent experience with arterial switch repair in the neonate with transposition of the great arteries (S,D,D) prompted repair in these patients by arterial switch, ventricular septal defect closure, repair of aortic arch obstruction, and augmentation of the right ventricular outflow tract.