Prenatally diagnosed pseudoaneurysm of mitral-aortic intervalvular fibrous area.

The mitral-aortic intervalvular fibrosa (MAIVF) is an avascular, fibrous structure that provides continuity between the anterior leaflet of the mitral valve and the aortic valve. Pseudoaneurysm of the MAIVF is rare and has been most commonly described in adults and, more rarely, in children following cardiac surgery or endocarditis. Few reports have been published on cases with congenital pseudoaneurysm of the MAIVF. Here, we describe five cases of congenital pseudoaneurysm of the MAIVF identified prenatally and an additional six cases diagnosed postnatally. This is an unusual finding of varying clinical significance, which can be isolated or associated with complex congenital heart disease but, importantly, can be identified and monitored in the fetus. © 2022 International Society of Ultrasound in Obstetrics and Gynecology.

Severe left diaphragmatic hernia limits size of fetal left heart more than does right diaphragmatic hernia.

OBJECTIVES: To assess whether severity of congenital diaphragmatic hernia (CDH) correlates with the degree of left heart hypoplasia and left ventricle (LV) output, and to determine if factors leading to abnormal fetal hemodynamics, such as compression and reduced LV preload, contribute to left heart hypoplasia. METHODS: This was a retrospective cross-sectional study of fetuses at 16-37 weeks' gestation that were diagnosed with CDH between 2000 and 2010. Lung-to-head ratio (LHR), liver position and side of the hernia were determined from stored ultrasound images. CDH severity was dichotomized based on LHR and liver position. The dimensions of mitral (MV) and aortic (AV) valves and LV were measured, and right and left ventricular outputs were recorded. RESULTS: In total, 188 fetuses with CDH were included in the study, 171 with left CDH and 17 with right CDH. Fetuses with severe left CDH had a smaller MV (Z = -2.24 ± 1.3 vs -1.33 ± 1.08), AV (Z = -1.39 ± 1.21 vs -0.51 ± 1.05) and LV volume (Z = -4.23 ± -2.71 vs -2.08 ± 3.15) and had lower LV output (26 ± 10% vs 32 ± 10%) than those with mild CDH. MV and AV in fetuses with right CDH (MV, Z = -0.83 ± 1.19 and AV, Z = -0.71 ± 1.07) were larger than those in fetuses with left CDH, but LV outputs were similarly diminished, regardless of hernia side. Severe dextroposition and abnormal liver position were associated independently with smaller left heart, while LHR was not. CONCLUSION: The severity of left heart hypoplasia correlates with the severity of CDH. Altered fetal hemodynamics, leading to decreased LV output, occurs in both right- and left-sided CDH, but the additional compressive effect on the left heart is seen only when the hernia is left-sided. Improved knowledge of the physiology of this disease may lead to advances in therapy and better risk assessment for use in counseling affected families.

Improved surgical outcome after fetal diagnosis of hypoplastic left heart syndrome.

BACKGROUND: Hypoplastic left heart syndrome (HLHS) is frequently diagnosed prenatally, but this has not been shown to improve surgical outcome. METHODS AND RESULTS: We reviewed patients with HLHS between July 1992 and March 1999 to determine the influence of prenatal diagnosis on preoperative clinical status, outcomes of stage 1 surgery, and parental decisions regarding care. Of 88 patients, 33 were diagnosed prenatally and 55 after birth. Of 33 prenatally diagnosed patients, 22 were live-born, and pregnancy was terminated in 11. Of 22 prenatally diagnosed patients who were live-born, 14 underwent surgery, and parents elected to forego treatment in 8. Of 55 patients diagnosed postnatally, 38 underwent surgery, and 17 did not because of parental decisions or clinical considerations. Prenatally diagnosed patients were less likely to undergo surgery than patients diagnosed after birth (P:=0.008). Among live-born infants, there was a similar rate of nonintervention. Among patients who underwent surgery, survival was 75% (39/52). All patients who had a prenatal diagnosis and underwent surgery survived, whereas only 25 of 38 postnatally diagnosed patients survived (P:=0.009). Patients diagnosed prenatally had a lower incidence of preoperative acidosis (P:=0.02), tricuspid regurgitation (P:=0.001), and ventricular dysfunction (P:=0.004). They were also less likely to need preoperative inotropic medications or bicarbonate (P:=0.005). Preoperative factors correlating with early mortality included postnatal diagnosis (P:=0.009), more severe acidosis (P:=0.03), need for bicarbonate or inotropes (P:=0.008 and 0.04), and ventricular dysfunction (P:=0.05). CONCLUSIONS: Prenatal diagnosis of HLHS was associated with improved preoperative clinical status and with improved survival after first-stage palliation in comparison with patients diagnosed after birth.

Malaligned outlet septum with subpulmonary ventricular septal defect and abnormal ventriculoarterial connection: a morphologic spectrum defined echocardiographically.

A retrospective analysis of 62 echocardiograms from 22 patients with malaligned outlet septum, subpulmonary ventricular septal defect and abnormal ventriculoarterial (VA) connection was performed and the results were confirmed by intraoperative inspection, cardiac catheterization and angiography, or pathologic specimen analysis in 21 of the 22 patients. The purpose of this study was to determine the relative incidence of the primary morphologic features; the correlation between the presence or absence of mitral valve to pulmonary valve fibrous continuity and the type of VA connection; the types and incidence of additional cardiovascular anomalies; and the correlation between the determination of the VA connection by echocardiography versus that ascertained at intraoperative and autopsy inspection. A spectrum of VA connections was found, ranging from double outlet right ventricle (23%) at one extreme, through an intermediate type (64%) in which the pulmonary artery was overriding the ventricular septum, thereby having no consistent and predominant connection to either ventricle, to a discordant VA connection (13%) at the opposite end of the spectrum. All three types of VA connection occurred whether or not there was mitral valve to pulmonary valve fibrous continuity. Additional cardiovascular anomalies were present in 95% of patients, the most common being aortic arch obstructive lesions in 59%, anomalous attachments of the atrioventricular valve tension apparatus in 55% and subaortic stenosis in 50%. A method of echocardiographic determination of the VA connection that correlated with that determined intraoperatively and from pathologic specimen analysis is described.

Echocardiographic spectrum of double inlet ventricle: evaluation of the interventricular communication.

To determine the spectrum and associated anomalies of double inlet ventricle (single ventricle), echocardiographic data of 50 patients with double inlet ventricle were reviewed and compared with the data obtained by cardiac catheterization, cardiac surgery and autopsy. Standard echocardiographic planes were used to determine the cardiac anatomy and the size of the interventricular communication. Double inlet by way of two perforate valves was found in 44 patients. In 42 of the 44 patients the dominant ventricular morphology was of the left ventricular type (double inlet left ventricle); in 13 of these 42 patients stenosis of one atrioventricular (AV) valve was found. Double inlet right ventricle and double inlet indeterminate ventricle were each found in one patient. Double inlet by way of a common AV valve was found in six patients, all of whom had atrial isomerism. The diagnosis of double inlet ventricle was accurate by two-dimensional echocardiography in all 44 patients. A restrictive interventricular communication was shown in 13 patients and a nonrestrictive communication in 17 patients by cardiac catheterization. Patients with a restrictive interventricular communication had a significantly smaller interventricular communication area normalized by the body surface area (mean +/- SD 1.21 +/- 0.53 cm2/m2) than did those with a nonrestrictive interventricular communication (2.33 +/- 0.71 cm2/m2) (p less than 0.01). Infants with an aortic anomaly had a significantly smaller interventricular communication area (1.35 +/- 0.65 cm2/m2) than did those without an aortic anomaly (2.57 +/- 0.76 cm2/m2) (p less than 0.05). Echocardiography provides an accurate noninvasive diagnosis in patients with double inlet ventricle, offering reliable information about the restrictive interventricular communication.

Ebstein's anomaly: echocardiographic and clinical features in the fetus and neonate.

To determine which morphologic features are associated with early death, the complete echocardiograms and medical records of 16 consecutive patients with Ebstein's anomaly and concordant atrioventricular connections who presented in the fetal (n = 5) or neonatal (n = 11) period were reviewed. The cohort was classified into two groups on the basis of survival at 3 months. Group 1 consisted of seven patients who died at less than or equal to 3 months of age, and Group 2 consisted of the nine surviving patients. Comparing Groups 1 and 2, the respective incidence rates of morphologic features that correlated with early death (p less than 0.05) included tethered distal attachments of the anterosuperior tricuspid leaflet (86% versus 11%), right ventricular dysplasia (86% versus 0%), left ventricular compression by right heart dilation (71% versus 11%) and the area of the combined right atrium and atrialized right ventricle being greater than the combined area of the functional right ventricle, left atrium and left ventricle (57% versus 0%) measured in the apical four chamber view. Right ventricular dysplasia was present in all patients with marked right atrial and atrialized right ventricular enlargement, in 86% of patients with tethered anterior leaflets and in 83% of those with left ventricular compression; 86% of patients with right ventricular dysplasia had tethered distal attachments. In conclusion, echocardiography defines morphologic features in the fetus and neonate that are highly predictive of death by 3 months of age.

Conditions with right ventricular pressure and volume overload, and a small left ventricle: "hypoplastic" left ventricle or simply a squashed ventricle?

OBJECTIVES: We modeled the utility of preoperative potential left ventricular (LV) volume in predicting postoperative volume in conditions causing LV compression. BACKGROUND: With right ventricular (RV) overload lesions, LV "hypoplasia" may be primarily due to compression by reverse septal bowing. If so, preoperative potential LV volume should correspond 1:1 with postoperative volume. The potential volume for a given endocardial circumference can be calculated from the maximal potential cross-sectional area (where A = circumference(2)/4pi) and LV length. METHODS: We studied echocardiographic variables from 22 patients with RV overload lesions perioperatively. RESULTS: Preoperative LV volume was 15.0 +/- 7.1 ml/m2 (59% of patients had a volume <15 ml/m2); potential volume was 20.0 +/- 9.8 ml/m2. Postoperative volume increased to 28.2 +/- 8.6 ml/m2 (100% of patients had a volume >15 ml/m2). Preoperative potential volume correlated well with, but generally underestimated, postoperative volume (r = 0.75, p < 0.0001). Postoperative increases in both LV circumference and length contributed to this discrepancy. CONCLUSIONS: In RV overload lesions, LV "hypoplasia" is primarily due not to compression; rather it is due to underfilling. Even "hypoplastic" ventricles can achieve an adequate cavity after operation normalizes loading conditions. Both true and potential preoperative volume can predict postoperative volume well. However, potential volume, which is less prone to underestimating ventricular adequacy, may better help to determine suitability for biventricular repair in lesions of RV overload associated with a "hypoplastic" LV.

Echocardiographic techniques for assessing normal and abnormal fetal cardiac anatomy.

Diagnostic quality images of the fetal heart in utero can be obtained as early as 18 to 20 weeks of gestation. The cardiac structures can be imaged primarily by cross-sectional echocardiography and augmented by a combination of simultaneous M-mode echocardiography and range-gated pulsed Doppler ultrasonography. Cross-sectional images from planes through the fetal heart equivalent to planes that can be obtained after birth can be generated in utero. In a study of 168 pregnancies, 10 structural cardiac abnormalities have been defined. These abnormalities and others that have been reported indicate the potential for in utero cardiac diagnosis. The recognition of structural congenital heart disease in utero has been helpful in genetic counseling, planning the method of labor and delivery and making decisions regarding termination of pregnancy and planning postnatal care. Fetal echocardiography offers the potential to change the pattern of health care delivery to those with suspected congenital heart disease.

Evaluation of the responsiveness of elevated pulmonary vascular resistance in children by Doppler echocardiography.

Changes in the Doppler indexes, acceleration time (AcT), right ventricular ejection time (RVET), AcT/RVET ratio and pulmonary artery peak velocity were measured as were changes in pulmonary artery pressure and pulmonary vascular resistance in 21 children with pulmonary hypertension due to a large interventricular communication. In 11 children pulmonary vascular resistance was greater than 4.6 U/m2 (mean 8.6 +/- 1.6), whereas in 10 it was less than 4.5 U/m2 (mean 3.4 +/- 0.2). Although both groups demonstrated acceleration time and AcT/RVET values above normal, there were no significant differences in these values between the groups with high and low pulmonary vascular resistance. With administration of a pulmonary vasodilator pulmonary vascular resistance decreased in 11 responders by greater than 50% of baseline values (from 5.3 +/- 0.7 to 1.6 +/- 0.3 U/m2), whereas in the 10 nonresponders mean pulmonary vascular resistance decreased from 7.0 +/- 1.9 to 4.9 +/- 1.1 U/m2. There was no significant change in the Doppler indexes except for an increase in pulmonary artery peak velocity in the responders from 1.34 +/- 0.07 to 1.66 +/- 0.06 m/s (p less than 0.001). The results indicate that Doppler echocardiography cannot predict either the level of increased pulmonary vascular resistance or the degree of responsiveness sufficiently to obviate the need for cardiac catheterization in patients with interventricular communication and pulmonary hypertension.

Contrast two-dimensional echocardiography in congenital heart disease: techniques, indications and clinical utility.

Saline contrast echocardiography was performed in 889 children from June 1976 through February 1988. One-third of these studies were in postoperative patients. A patent foramen ovale was identified by finding right to left shunting on venous contrast injection in 37% of 127 children studied with a structurally normal heart. The incidence of such shunting was greater at younger ages (55% younger than 1 month versus 22% older than 1 month). In most patients with an atrial or ventricular septal defect, some right to left shunting was demonstrable. The technique was useful in distinguishing different forms of atrial septal defect and identifying muscular ventricular septal defects that were difficult to image directly. The technique was used in the catheterization laboratory to aid in the identification of congenital coronary artery fistulas and was diagnostic in two cases of pulmonary arteriovenous malformation. In patients with situs abnormalities, the technique was useful in identifying the systemic venous connections to the atria. Contrast echocardiography was also used in postoperative evaluations. The technique was useful in identifying patch leaks and residual defects after Senning, Mustard and Fontan operations, and after closure of atrial and ventricular septal defects. Most patients were found to have no superior vena cava obstruction by contrast echocardiography after the Senning or Mustard procedure. Contrast echocardiography continues to be a useful technique in the diagnosis of a wide spectrum of congenital heart disease, as well as in the postoperative evaluation of congenital heart surgery.

Recognition of left coronary artery fistula to the left and right ventricles by contrast echocardiography.

A coronary-cameral fistula was inspected clinically by two-dimensional and pulsed Doppler ultrasound. At cardiac catheterization a fistulous connection between the left coronary artery and the right ventricle was observed. Contrast echocardiography using agitated saline solution injected into the aortic catheter clearly showed the passage of microcavitations into the left and the right ventricles confirming the connection of the fistula to both chambers. Contrast echocardiography is a valuable technique that can help define the site of drainage of coronary artery fistulas.

Fibrous obstruction within the left ventricular outflow tract associated with ventricular septal defect: a pathologic study.

OBJECTIVES: We examined the nature of ridges within the left ventricular outflow tract associated with ventricular septal defects that might be found by echocardiography. BACKGROUND: Echocardiography displays even small ridges well. Surgical removal of such ridges at the time of defect closure is recommended. METHODS: We examined 37 heart specimens with ventricular septal defects with a ridge, noting its nature and relation to the defect and adjacent valves. We excluded left ventricular outflow tract obstruction associated with complex lesions. RESULTS: Defects were perimembranous in 25 specimens, muscular in 8 and part of an atrioventricular septal defect in 5. Some hearts had multiple defects. Many of the original reports had not mentioned ridges. Three distinct ridge patterns were found. The first (n = 18) was a fold of endocardial tissue related to the membranous septum. The second (n = 12) was a defect of a fibrous nature; in 8 this was a discrete, protuberant fibrous ridge, and in 4 the obstruction was diffuse, which we termed keloidal. The third pattern (n = 7) lay circumferentially around the ventricular septal defect, seemingly associated with the defect's attempted spontaneous diminution in size. Endocardial folds were not found in specimens from patients > 5 years old. Fibrous and keloidal lesions, which may represent a continuum of progression, generally were found in specimens from older patients. Histologic studies of 17 specimens confirmed the morphologic findings. The endocardial folds were endothelial tissue, whereas the fibrous and keloidal ridges were of fibrous tissue, as were circumferential lesions. All specimens had mitral-semilunar valvular continuity. CONCLUSIONS: Endocardial fold and circumferential lesions appear to be benign. The endocardial folds arose from the membranous ventricular septum, were not protuberant and usually were found in younger patients. The fibrous ridges, in contrast, were protuberant and were always associated with the underlying muscle of the outlet septum. These pathologic distinctions may facilitate echocardiographic diagnosis and prognosis.

Determination of pulmonary to systemic blood flow ratio in children by a simplified Doppler echocardiographic method.

Doppler echocardiographic determination of the pulmonary to systemic blood flow (Qp/Qs) ratio requires calculation of pulmonary artery and aortic luminal areas and integrals of the Doppler systolic velocity curves. To simplify the Doppler calculation of the Qp/Qs ratio, the square of the ratio of pulmonary to aortic luminal diameters, substituted for the ratio of vessel areas, was multiplied by the ratio of pulmonary to aortic peak flow velocities, substituted for the ratio of velocity time integrals. The Qp/Qs ratios were calculated by both the conventional and the simplified Doppler method in 42 children, 1 month to 16 years of age. Fifteen children had no evidence of shunt and 27 had an intracardiac left to right shunt at the atrial or ventricular level, or both. In children with a shunt, the Qp/Qs ratio obtained at cardiac catheterization by oximetry ranged from 1.4:1 to 4.3:1. Both the conventional and the simplified Doppler Qp/Qs ratios correlated well with the oximetric Qp/Qs ratios (r = 0.94 and 0.93, respectively). Since the pulmonary to aortic luminal area ratio and velocity time integral ratio varied in each patient, neither measurement alone correlated well with the oximetric Qp/Qs ratio and, therefore, neither alone could be used as an estimate of the Qp/Qs ratio. The simplified Doppler calculation of the Qp/Qs ratio, which requires less time and no computer facilities, provides an excellent estimate of the Qp/Qs ratio in children.

Pulmonary artery origin of the left coronary artery: diagnosis by two-dimensional echocardiography, pulsed Doppler ultrasound and color flow mapping.

Five children, aged 0.2 to 6.7 years, with pulmonary artery origin of the left coronary artery proved by angiography underwent echocardiographic examination from 1985 through 1986. Prospective identification of this condition was achieved by two-dimensional echocardiography alone in two patients. Using a parasagittal plane from the second or third intercostal space, identification of the anomalous origin from the pulmonary artery was also possible in the other children on subsequent examination. In all of these studies the right coronary artery was considerably enlarged. The ratio of the diameters of the right coronary artery and the aortic root was 0.25 to 0.33 (normal 0.12 +/- 0.02). Flow mapping by color Doppler ultrasound in three children studied most recently and peripheral arterial or aortic root contrast echocardiography identified the site of entry of the coronary flow into the pulmonary artery. The flow disturbance was detected on the aortic surface of the pulmonary artery opposite the portion from where the flow from a patent ductus is usually identified. Pulsed Doppler ultrasound identified the flow disturbance in the pulmonary artery in only three of the children. The two youngest patients did not demonstrate turbulence on pulsed Doppler ultrasound, but they were not examined by color Doppler ultrasound. The accuracy of noninvasive detection of pulmonary artery origin of the left coronary artery by ultrasound is improved by the use of a combined echocardiographic approach.

Changes of right ventricular size and function in neonates after valvotomy for pulmonary atresia or critical pulmonary stenosis and intact ventricular septum.

Right ventricular end-diastolic and stroke volumes were calculated from orthogonal subcostal echocardiographic images in 24 neonates (mean weight +/- SD 3.4 +/- 0.4 kg) with pulmonary atresia (n = 18) or critical pulmonary stenosis (n = 6) and intact ventricular septum before and at an average of 5 days and then 19 days after pulmonary valvotomy. The preoperative echocardiographic volume determinations were compared with the respective angiographic determinations. In addition, the endocardial area outlines of the left and right ventricles were obtained by planimetry from an end-diastolic frame taken in the apical four-chamber view. End-diastolic and stroke volumes calculated by the echocardiographic method (y) correlated closely with those calculated by the angiographic method (x); the regression equations were y = 1.02 x -0.13 (r = 0.95, SEE +/- 0.45 ml) and y = 1.16 x -0.15 (r = 0.89, SEE +/- 0.38 ml), respectively. All except one infant had right ventricular hypoplasia before valvotomy with an end-diastolic volume of 16.6 +/- 6.4 ml/m2 (44.5 +/- 17.3% of normal). Right to left ventricular area ratio was 0.56 +/- 0.09 (normal 0.95). Five days after valvotomy, right ventricular end-diastolic volume decreased to 10.6 +/- 4.6 ml/m2 (p less than 0.05) and stroke volume decreased from 8.3 +/- 3.5 to 5.5 +/- 2.8 ml/m2 (p less than 0.05). Nineteen days after valvotomy, right ventricular end-diastolic volume and right to left ventricular area ratio had increased to their respective preoperative values; right ventricular stroke volume had increased further to 10.4 +/- 3.9 ml/m2 (p less than 0.05).(ABSTRACT TRUNCATED AT 250 WORDS)

Is a cleft in the anterior leaflet of an otherwise normal mitral valve an atrioventricular canal malformation?

OBJECTIVES: This study sought to ascertain the surgical anatomy of a cleft in the left atrioventricular (AV) valve. BACKGROUND: Important morphologic differences exist between hearts with a cleft in the anterior leaflet of an otherwise normal mitral valve and those with a so-called cleft in the left AV valve when there is an AV septal defect, but it has been customary to link the lesions together on developmental grounds. METHODS: Eight autopsied specimens with a cleft in the aortic (or anterior) leaflet of the mitral valve were studied in detail, and echocardiograms from 21 patients with such a cleft were compared with the specimens and with findings typical of the so-called partial AV canal and other forms of AV septal defect. RESULTS: The structure and direction of the cleft, location of the papillary muscles within the left ventricle and AV junctional morphology of hearts with an otherwise normally structured mitral valve were significantly different from typical findings in hearts with AV septal defects. CONCLUSIONS: It is necessary to distinguish morphologically a cleft in an otherwise normally structured mitral valve in hearts with separate right and left AV junctions from the trifoliate left component of a common AV valve in hearts with an AV septal defect and a common AV junction because the disposition of the AV conduction tissues varies markedly between the lesions.

The levoatriocardinal vein: morphology and echocardiographic identification of the pulmonary-systemic connection.

OBJECTIVES: This study considers the array of pulmonary-systemic connections made by the levoatriocardinal vein. The primary and associated lesions that play a role in forming this vein are examined, and echocardiography is discussed as a method for its rapid identification. BACKGROUND: The levoatriocardinal vein is a pulmonary-systemic connection that provides an alternative egress for pulmonary venous blood in left-sided obstructive lesions. It is thought to result from the persistence of anastomotic channels that connect the capillary plexus of the embryonic foregut to the cardinal veins. Only 12 cases of levoatriocardinal vein have been reported since its first description in 1926. A comprehensive description of the morphology and echocardiographic identification of this lesion has been unavailable because of its rarity. METHODS: A retrospective study was performed in 13 patients with a levoatriocardinal vein from the University of California, San Francisco. Echocardiographic findings were compared with those obtained by angiography or at necropsy. In addition, the details of 12 previously published case reports were reviewed. Age at presentation, primary obstruction to pulmonary venous return, integrity of the atrial septum and origin and drainage of the levoatriocardinal vein were compared. RESULTS: Patient age at presentation was < 2 years, with most patients presenting before age 6 months. Variations of the hypoplastic left heart syndrome accounted for the majority of primary defects encountered, although multiple but less severe left-sided lesions were seen. The atrial septum was functionally intact in most patients. The levoatriocardinal vein, defined echocardiographically, originated predominantly from the smooth-walled left atrium and drained to the superior vena cava or innominate vein; however, variations of this pattern existed. CONCLUSIONS: As a physiologic entity, the levoatriocardinal vein provides a mechanism for decompression of pulmonary venous return primarily in patients with left ventricular inflow obstruction. A levoatriocardinal vein is thought to form when the atrial septum fails to provide an alternate egress for left atrial blood. However, when a septal defect or alternative shunt occurs in conjunction with a levoatriocardinal vein, the clinical presentation may be postponed. Echocardiography provides a rapid, noninvasive modality for identifying the pulmonary-systemic connection, which may masquerade as the vertical vein in anomalous pulmonary venous connection or act as an occult source of left to right shunting in patients undergoing surgery for hypoplastic left heart syndrome.

Doubly committed subarterial ventricular septal defects: echocardiographic features and surgical implications.

Doubly committed subarterial (supracristal, subpulmonary) ventricular septal defects are often complicated by aortic regurgitation resulting from aortic valve herniation into the defect. The clinical, echocardiographic and catheterization findings in 48 patients aged 0.3 to 46.4 years (median 9.5) with a doubly committed subarterial ventricular septal defect were reviewed. Aortic valve herniation was present in 38 (79%) and 55% of these had aortic regurgitation. The prevalence of both findings increased gradually with advancing age. The defect was closed surgically in 41 patients. Surgery during the first 2 years of life (median 0.4 year) was performed in 13 patients (group I), mainly because of a large shunt with a pulmonary to systemic flow ratio (Qp/Qs) 3.8 +/- 1.4 (mean +/- SD). Aortic regurgitation was present preoperatively in two patients (15%), persisted postoperatively in one patient and did not develop in any after repair (median duration of follow-up 2.3 years, range 0.1 to 7.4). In the other 28 patients (group II) surgery was performed between 4.8 and 46.4 years of age (median 11.5). These patients were generally less symptomatic and had a smaller shunt (Qp/Qs 1.5 +/- 0.5, p less than 0.001). Preoperative aortic regurgitation was present in 18 (64%). It persisted in 15 postoperatively, but in 13 of these it had diminished. Two-dimensional echocardiography in multiple views identified the site of the ventricular septal defect in all patients. Serial echocardiographic examinations demonstrated the progressive nature of aortic valve herniation, the partial occlusion of the defect by the herniated sinus and the development of aortic regurgitation. These findings suggest that timely surgical closure of these defects may prevent aortic regurgitation.

Accessory and anomalous atrioventricular valvar tissue causing outflow tract obstruction: surgical implications of a heterogeneous and complex problem.

OBJECTIVES: The purpose of this study was to determine the effect of accessory or anomalous atrioventricular valvar apparatus on relief of outflow tract obstruction. BACKGROUND: Outflow tract obstruction due to accessory tissue or anomalous attachments of the atrioventricular valvar apparatus is an unusual but well-recognized problem. In addition to obstruction, anomalous attachments of the atrioventricular valvar apparatus may interfere with procedures to relieve outflow tract obstruction or perform outflow tract reconstruction. METHODS: Since 1992, we have operated on 21 patients (median age 4 years) with systemic (n=13), pulmonary (n=5) or bilateral (n=3) outflow tract obstruction due to accessory atrioventricular valvar tissue and/or anomalous attachments of the subvalvar apparatus. Primary diagnoses were isolated obstruction of the systemic outflow tract or aortic arch (n=7), transposition complexes (n=6), previously repaired atrioventricular septal defect (n=3), functionally single ventricle (n=3) and ventricular septal defect with pulmonary outflow obstruction (n=2). Outflow tract gradients ranged from 20-110 mm Hg (median 58 mm Hg). RESULTS: Complete relief of obstruction due to atrioventricular valvar anomalies was possible in 14 patients. In six patients, the planned procedure either had to be modified or only partial relief of the obstruction was achieved. In the remaining patient, who had borderline functionally single ventricle heart disease (unbalanced atrioventricular septal defect) and systemic outflow obstruction due to accessory and functional valvar apparatus, support was withdrawn because the parents refused univentricular palliation and the valvar anomalies precluded a Ross-Konno procedure. There were two early deaths. At follow-up ranging from 1 to 66 months (median 27 months), there was one death, and there has been no recurrence of outflow tract obstruction or residual atrioventricular valvar tissue. CONCLUSIONS: Outflow tract obstruction caused by accessory or anomalous atrioventricular valvar structures is an uncommon and heterogeneous group of conditions that can have significant surgical implications. In the majority of cases, tailoring of surgical techniques will permit complete relief of obstruction. However, such anomalies may limit standard surgical options and necessitate an innovative approach in some patients.

Diagnosis of coronary artery fistula by two-dimensional echocardiography, pulsed Doppler ultrasound and color flow imaging.

Ten consecutive patients with a coronary artery fistula, aged 1 day to 4 years, were studied by two-dimensional echocardiography, pulsed Doppler ultrasound and color flow imaging. All patients underwent cardiac catheterization, and seven patients had surgical closure of the fistula. The origin, course and site of drainage of the coronary artery fistula were correctly identified prospectively by echocardiographic examination in all patients. Color flow imaging was particularly helpful in visualizing the site of drainage of the fistula. Diameters of the right and left coronary arteries at their origin and of the aortic root were measured from two-dimensional echocardiographic frames and compared with measurements obtained in normal children. The ratio of coronary artery diameter to aortic root diameter in normal children was 0.14 +/- 0.03 (mean +/- SD) for the right coronary artery and 0.17 +/- 0.03 for the left coronary artery. These normal ratios were greatly exceeded for coronary arteries feeding the fistula, and ranged from 0.68 to 0.84 for the right coronary artery and from 0.34 to 0.52 for the left coronary artery. All anatomic information needed for surgical treatment of coronary artery fistula was consistently obtained by echocardiography with color flow imaging. The fistula was closed from within the heart in five patients and by ligation from the epicardial surface in two patients. In these latter patients, intraoperative color flow imaging at the time of ligation proved to be extremely valuable in achieving complete closure.