Comparative response of right and left ventricles to volume overload.

The cardiac volume data of 49 normal children were compared with those of 23 with secundum atrial septal defect and 24 with patent ductus arteriosus. Significantly smaller ventricular end-diastolic volumes were observed in the normal infants than in older children (right ventricle 53.9 versus 75.5 cm3/m2; left ventricle 46.7 versus 63.6 cm3/m2). "Distensibility" of the right ventricle (DRV), left ventricle (DLV) and left atrium increased normally with age. DRV and DLV were similar shortly after birth; thereafter, DRV increased more rapidly than DLV (mean DRV 12.7; mean DLV 7.8 cm3/m2 per mm Hg, P less than 0.001). In both atrial septal defect and patent ductus arteriosus, the ipsilateral (involved) ventricles had increased volume, increased output, normal ejection fraction and increased distensibility. The contralateral (left) ventricle in atrial septal defect was smaller than normal (39.6 versus 49.7 cm3, P less than 0.001), and had a smaller ejection fraction (0.63 versus 0.71, P less than 0.01) and output (3.70 versus 4.57 liters/min per m2, P less than 0.005). In contrast, the contralateral (right) ventricle in patent ductus arteriosus remained normal. Left atrial maximal volume was larger than normal in atrial septal defect (46.6 versus 35.9 cm3/m2, P less than 0.001). The left atrial and left ventricular volumes in patent ductus arteriosus were, respectively, 152 and 142 percent of normal, indicating comparable response to the volume load. The left head changes in atrial septal defect may be related both to a functionally restrictive defect and to the difference in distensibility of the ventricles.     

Ultrasonic contrast studies for the detection of cardiac shunts

Contrast echocardiography has achieved importance in the diagnosis of cardiac shunt lesions. The technique provides information about flow patterns and serves as an adjunct to identifying communications that may be too small to image, even with high resolution real time scanning. This report reviews clinical applications and experiences in the use of standard, peripherally injected echocardiographic contrast agents for the detection of atrial septal defect, ventricular septal defect and patent ductus arteriosus. The importance and development of transpulmonary contrast agents capable of crossing the pulmonary capillary bed to opacify the left ventricle are reviewed and experience with a variety of experimental echocardiographic contrast agents is presented. Agents opacifying the left ventricle after intravenous injection are capable of providing direct ultrasonic contrast imaging of congenital left to right shunts. Further, recent experience with an experimental standardized, gas-producing contrast agent in an open chest animal model with an experimentally produced ventricular septal defect suggests that a combination of an experimental right heart agent that produces a measurable and reproducible amount of contrast effect, with a videodensitometric system capable of quantifying both positive and negative contrast effects, may provide an ultrasonic method for evaluating the magnitude of cardiac shunts.     

Transcatheter occlusion of ventricular septal defect

The patent ductus arteriosus occlusion device (Rashkind-USCI) was employed to occlude a residual ventricular septal defect after Fontan procedure in a patient with hypoplastic right ventricle, ventricular septal defect, and pulmonary stenosis. There was significant right-to-left shunting across the ventricular septal defect, with cyanosis exacerbated by exercise. After placement of the 17 mm occlusion device, the right-to-left shunt was markedly diminished, and the cyanosis resolved. Occlusion of ventricular septal defects in patients with complex congenital heart disease may be performed with the patent ductus arteriosus occluder in selected instances.     

Transarterial pulmonary venous wedge arteriography in pulmonary atresia,ventricular septal defect,and intact atrial septum

Transarterial pulmonary venous wedge arteriography was performed in four cases of pulmonary atresia (PA), ventricular septal defect (VSD), and intact atrial septum. In two cases, only the left pulmonary artery was visualized by selective injection to the patent ductus arteriosus (PDA) or Blalock-Taussig anastomosis (B-T shunt), and the right pulmonary artery was opacified by transarterial retrograde pulmonary arteriography. In two other cases, with nonfunctioning B-T shunt, the pulmonary artery was not opacified by either aortography nor by injection into the B-T shunt. Transarterial pulmonary vein wedge arteriography visualized a confluent pulmonary artery. Transarterial pulmonary vein wedge arteriography was a useful and safe procedure for opacifying the pulmonary artery that could not be visualized antegradely in VSD, PA, and intact atrial septum.     

Mitral Atresia with Hypoplastic Left Ventricle and Multiple Shunt Lesions

Mitral atresia is a rare congenital heart defect when compared to atresia of other valves and it is often associated with other cardiac malformation such as patent ductus arteriosus, coarctation of the aorta, transposition of the great arteries, pulmonary stenosis, shunt defects, cor triatriatum, chamber, and valvular anomalies. There have been few reports of mitral atresia, therefore, the case of a 5-month-old male child with complaint of difficulty with breathing and echocardiographic diagnosis of atretic mitral valves, atrial septal defect of 15 mms, ventricular septal defect (VSD) of 10 mms and hypoplastic left ventricle is reported.     

Supravalvular stenosing ring of the left atrium associated with corrected transposition of the great vessels

A case of supravalvular stenosing ring of the left atrium is described. The severity of the stenosis obscured the features of Ebstein's malformation of the left atrioventricular valve, which was the only other defect associated with corrected transposition. Analysis of the reported examples of supravalvular stenosing rings reveals that the condition rarely exists as an isolated anomaly but tends to occur in 2 groups of conditions, namely, a developmental complex of obstructive anomalies of the left side of the heart (supravalvular ring, subaortic stenosis, parachute mitral valve and coarctation of the aorta) or a second group associated with a left to right shunt produced by a ventricular septal defect or patent ductus arteriosus. The potential diagnostic value of echocardiography in cases in which there is evidence of pulmonary venous obstruction is discussed.     

Heart malformations in trisomy 13 and trisomy 18]

Congenital heart diseases were studied in children diagnosed of trisomy 13 and trisomy 18 in our hospital between January 1973 and July 1990. Twenty patients with trisomy 18 were diagnosed (18 females and two males). All had cardiac malformations. The findings were: ventricular septal defect in 16 cases (80%), valvular anomalies in 12 (63%), patent ductus arteriosus in nine (47%) and atrial septal defect or patent foramen ovale in 7 cases (36%). We found some complex congenital cardiac diseases: one atrioventricular canal, one tetralogy of Fallot, one hypoplastic left ventricle with mitral atresia and double outlet right ventricle, one case of univentricular heart with aortic outlet from a rudimentary cavity, a right ventricular atresia with pulmonary and tricuspid valves atresia. Nine cases of trisomy 13 were diagnosed (seven females and two males). We found: ventricular septal defect in 7 cases (77%), valvular disease in five (100% of the necropsy studies), secundum atrial septal defect in 4 patients (80%) and patent ductus arteriosus in two. Two cases presented hypoplastic left ventricle with aortic arch hypoplasia, one of them had subaortic stenosis and left superior vena cava being connected to the right atrium via coronary sinus; one case showed fibroelastosis. Our results have been similar to the previously reported and confirm the invariably presence of cardiac malformations in these syndromes. These malformations are an important sign of suspicion in fetal ultrasonography.     

Clinical problems of postoperative pulmonary vascular disease

The younger the patient with a large left to right shunt at the time of operation, the greater the likelihood that pulmonary vascular resistance will fall to normal thereafter. In older patients, the degree to which the pulmonary vascular resistance is elevated before operation is a critical factor determining operability and prognosis. Patients at particularly high risk for the development of significant pulmonary vascular obstruction early in life are those with certain forms of cyanotic congenital heart disease, such as complete transposition of the great arteries with ventricular septal defect and patent ductus arteriosus, and truncus arteriosus. Other conditions in which pulmonary vascular obstruction appears to progress rapidly include large ventricular septal defect, complete atrioventricular canal defect and left to right shunt lesions in an environment of high altitude or associated with unilateral pulmonary arterial absence of the Down's syndrome. In this report the framework is reviewed for recognizing that important pre- and postnatal modifiers of the pulmonary vascular bed may be lesion-dependent. Thus, the growth and development of the pulmonary vascular bed during fetal and early postnatal life, as well as the morphologic alterations described in detail by Heath and Edwards, are likely to determine the ultimate intensity and magnitude of pulmonary vascular obstruction. Commentary is also provided concerning the management of patients with high pulmonary vascular resistance during pregnancy and delivery, their response to exercise, and the possibility of medical treatment designed to reduce pulmonary vascular resistance, and perhaps prolong life and enhance its quality.     

彩色多普勒超声心动图诊断完全性大动脉转位

目的 探讨彩色多普勒超声早期诊断完全性大动脉转位的价值。方法 采用彩色超声检查完全性大动脉转位80例,按顺序分段法进行。结果 80例均显示主动脉在前,发自右心室,肺动脉在后,发自左心室,合并畸形包括VSD、ASD、PDA、PS等。其中2例手术病例及7例尸解病例与超声诊断相符。结论 超声心动图能准确诊断大动脉转位及伴随畸形,并提供部分血流动力学资料。     

New serial contrast technique for assessment of left to right shunting patent ductus arteriosus in the neonate

A new serial echocardiographic contrast technique for detection of patent ductus arteriosus has been developed and validated by clinical course (33 infants), surgical observations (13 infants) and autopsy observations (4 infants). A left to right shunting patent ductus arteriosus was demonstrated in 30 of 33 prospectively studied premature infants (mean weight 1,371 g) using this new contrast method. The demonstration of left to right ductal shunting was accomplished by hand injection of a nonviscous material (saline solution, 5 percent dextrose in water or the patient's own blood) through an umbilical arterial catheter placed with its tip located above the diaphragm during recording of a suprasternal notch echocardiogram. In positive studies, both the transverse aortic arch and right pulmonary artery were opacified. If no left to right shunt existed, only the transverse aortic arch was opacified.This serial study helped elucidate the natural history of patent ductus arteriosus. Twenty-four of the 30 patients with a positive study initially had a left to right shunting patent ductus arteriosus without an audible murmur. Three of these patients with silent patent ductus arteriosus later had congestive heart failure and two required operative ligation of the patent ductus. The high rate of detection of silent patent ductus arteriosus and its bedside confirmation when suspected are important in the serial management of critically ill newborns with this condition. The test is safe and sensitive and it is useful for early demonstration of silent patent ductus arteriosus, for clarifying the origin of murmurs and for confirming spontaneous, operative or pharmacologie closure of a patent ductus arteriosus.     

Hypertensive pulmonary vascular disease in alpha-transposition of the great arteries

In subjects with classic complete transposition (d-transposition) of the great arteries, the pulmonary arterial plexiform lesion, characteristic of chronic high levels of pulmonary arterial pressure, was observed in those with an intact ventricular septum as well as in those with a ventricular septal defect. The lesion was not observed before age 12 months. Among 16 patients aged 12 to 30 months, the plexiform lesion was observed in 6 of 12 patients (50 percent) with an intact ventricular septum and a closed ductus arteriosus and in 1 of 4 patinets with an associated ventricular septal defect. The basis of the plexiform lesion, which is considered a sign of chronic severe pulmonary hypertension, occurring in subjects with an intact ventricular septum and a closed ductus arteriosus is not explained. The phenomenon observed in this study confirms the earlier observations of others.     

Pulmonary vascular disease in complete transposition of the great arteries: A study of 200 patients

Lung specimens of 200 patients with transposition of the great arteries were examined microscopically for evidence of pulmonary vascular disease. In patients with an intact ventricular septum or a small ventricular septal defect, advanced pulmonary vascular disease was uncommon; only 9 of 107 such patients (8.4 percent) demonstrated greater than grade 2 (Heath-Edwards) pulmonary vascular disease. A persistent large patent ductus arteriosus appeared to promote progressive pulmonary vascular disease in this group since each of the five infants less than 1 year of age with grade 3 or 4 disease had this lesion. In contrast, pulmonary vascular disease was common in patients with a large ventricular septal defect; 37 of 93 patients (40 percent) with this defect had greater than grade 2 pulmonary vascular disease. Among patients more than 1 year of age, 26 of 35 (75 percent) had grade 4 disease. The catheterization data suggest that the calculated pulmonary vascular resistance may underestimate the degree of disease, probably by overestimating the pulmonary blood flow (Fick method). Pulmonic stenosis appeared to protect the lungs from progressive pulmonary vascular disease, and pulmonary arterial banding was protective when performed before age 6 months. Our studies indicate that a persistent large patent ductus arteriosus should be closed as early as possible in view of its association with advanced pulmonary vascular disease in these patients. In infants with a large ventricular septal defect, pulmonary arterial banding or corrective surgery with closure of the defect should be performed between the ages of 4 and 6 months to prevent progressive pulmonary vascular damage.     

Technical advances in the Senning operation

Since March 1978, 59 patients with transposition of the great arteries (TGA) ranging in age from 2 months to 6 years have undergone the Senning operation. Forty-six patients (77.9%) had transposition with intact ventricular septum, 8 of them with patent ductus arteriosus; 8 patients (13.5%) had TGA with ventricular septal defect (VSD), 3 of them with previously performed banding; 4 patients (6.7%) had TGA with VSD and pulmonary stenosis (PS), and one (1.6%) had TGA with PS. Prior to correction, 38 cases had undergone a Rashkind septotomy and 15 had had a previous Blalock-Hanlon septectomy. During this time we have introduced some technical modifications of the original Senning operation: (A) right atrial incision, (B) large left atrial wall window, (C) infrasinal suture of the left atrial flap, (D) avoidance of damage to the hypothetical anterior atrioventricular conduction bundle, and recently, in 12 patients, we have used (E) an inverted left atrial appendage as an interatrial flap. There were 9 hospital deaths (15.2%) caused by low cardiac output (4 cases), severe hypoxemia and low cardiac output (4 cases) and one patient with pulmonary venous drainage obstruction died following reoperation. Two patients developed neurological complications that disappeared without sequela. Another complication was a right chylothorax (4 patients), which improved without the necessity of surgical repair. Eighteen patients developed a junctional rhythm for a period over 24 hours. We think that the technical modifications which we present here, partially contribute to improving the Senning operation.     

Echocardiographic evaluation of right ventricular function in complete transposition of the great arteries: angiographic correlates.

Right ventricular function of patients with complete transposition of the great arteries may be impaired. To assess whether the M mode echocardiogram could predict right ventricular function 64 echocardiograms and ventricular angiograms of 62 patients were studied, 33 obtained before repair of the defect (group I) and 31 (including 2 from patients in group I) obtained after the Mustard procedure (group II). Each group was subdivided according to whether a ventricular septal defect or patent ductus arterposus was or had been present preoperatively.The right ventricular preejection period was expressed as the ratio of the observed to the predicted value for a systemic left ventricle. This value was above normal In all groups and was not significantly different in the 13 patients with right bundle branch block. The observed ejection time expressed as a percent of that predicted for a systemic left ventricle correlated with right ventricular ejection fraction (r = 0.47). The ratio of the observed preejection period/ejection time had a higher correlation with right ventricular ejection fraction (r = ? 0.70). Nine patients had a low right ventricular ejection fraction (an ejection fraction of 0.55 or less was considered indicative of poor right ventricular function). These nine patients were all in group II and all had right bundle branch block.Most patients with poor right ventricular function had had a ventricular septal defect or patent ductus arteriosus before repair of the transposition, and postoperatively had angiographic tricuspld regurgitation, right bundle branch block, a preejection period/ejection time ratio greater than 0.55 and a percent predicted ejection time of 96 percent or less. Most patients who had good right ventricular function (ejection fraction greater than 0.55) had none of these findings. Systolic time intervals obtained with M mode echocardiography can be used for the assessment and longitudinal follow-up of right ventricular function in patients with transposition.     

Transcatheter left ventricular outflow tract stent placement in a low birth weight child with hypoplastic left ventricle,mitral atresia,transposition of the great arteries,ventricular septal defect and severe pulmonary stenosis

A baby boy, the first of triplets born at 33 weeks gestation weighing 1.88 kg, presented with neonatal cyanosis and was diagnosed with mitral atresia, hypoplastic left ventricle, ventricular septal defect, d‐transposition of the great arteries, severe pulmonary stenosis, and hypoplastic branch pulmonary arteries. He was treated with prostaglandin until seven weeks of age. The patent ductus arteriosus was curly Q and not suitable for stent placement. Cardiac catheterization was undertaken and using an antegrade approach, a Multi‐Link Ultra stent was implanted across the left ventricular outflow tract (LVOT) and inflated to 5 mm to improve antegrade pulmonary blood flow. This allowed reasonable pulmonary arterial growth to allow the patient undergo bidirectional Glenn shunt at five months of age. This first report of LVOT stenting in this setting may provide an alternative to placement of a systemic to pulmonary arterial shunt when ductal stenting is not possible. © 2013 Wiley Periodicals, Inc.     

First-pass radionuclide angiocardiography in the determination of left-to-right cardiac shunt site in children

To ascertain if the level of a left-to-right cardiac shunt can be reliably established by first-pass radionuclide angiocardiography with ^99mTc-pertechnetate, 102 children have been studied; 19 without a shunt, 26 with an atrial septal defect (ASD), 45 with a ventricular septal defect (VSD), and 12 with a patent ductus arteriosus (PDA). Time-activity curves were generated over the right atrium and ventricle, and several quantitative parameters were derived from the curves. It was concluded that in the absence of right-sided valvular incompetence, the time-activity curves show the greatest abnormality in the cardiac chambers distal to the left-to-right shunt. The right ventricular curve is typically altered in ASD but cannot be relied upon to differentiate VSD or PDA. The identification of ASD is assisted by the use of two of the parameters, the count ratio C4:C1 and time ratio T2:T1.     

Clinical utility of two-dimensional doppler echocardiographic techniques for estimating pulmonary to systemic blood flow ratios in children with left to right shunting atrial septal defect, ventricular septal defect or patent ductus arteriosus

Range gated two-dimensional Doppler echocardiographic methods were evaluated for quantifying pulmonary (QP) to systemic (QS) blood flow ratios. Twenty-one patients were studied, 4 with patent ductus arteriosus, 6 with atrial septal defect and 11 with ventricular septal defect. The Doppler pulmonary to systemic flow (QP:QS) estimation method involved calculating volume flow (liters/min) at a variety of intracardiac sites by using imaging information for flow area and Doppler outputs to calculate mean flow velocity as a function of time. Area volume flows were combined to yield QP:QS ratios. The sites sampled were main pulmonary artery, ascending aorta, mitral valve orifice and subpulmonary right ventricular outflow tract. The overall correlation between Doppler QP:QS estimates and those obtained at cardiac catheterization (n = 18) or radionuclide angiography (n = 3) was r = 0.85 (standard error of the estimate = 0.48:1). These preliminary results suggest that clinical application of this Doppler echocardiographic method should allow noninvasive estimation of the magnitude of cardiac shunts.     

Two-dimensional echocardiographic features of interruption of the aortic arch

The 2-dimensional echocardiographic features of interruption of the aortic arch are presented based on analysis of the echocardiograms and angiograms from 8 infants: 2 with type A and 6 with type B interruption. Each infant had a patent ductus arteriosus, 6 had a conoventricular septal defect with leftward deviation of the conal septum, 1 had truncus arteriosus with truncal valve stenosis, and 1 had a distal aortopulmonary septal defect with an intact ventricular septum. Echocardiographic images obtained from the suprasternal notch or from a high parasternal approach demonstrated the interruption of the aortic arch and continuation of the patent ductus arteriosus into the descending aorta. These findings were compared with those in infants with aortic atresia and a hypoplastic ascending aorta or discrete coarctation of the aorta with tubular hypoplasia of the aortic arch. Types A and B interruption of the aortic arch were easily differentiated and the caliber of the patent ductus arteriosus was assessed. The characteristic conoventricular septal defect was readily visualized from an apex 2-chamber view or from a subcostal sagittal plane view. With this information subsequent angiography can be more expeditiously performed in this group of critically ill infants.     

Ivemark syndrome in association with congenital septum transversum defect and pancreatic divisum.

A four-month-old female baby presented with cyanosis and respiratory distress. A provisional diagnosis of congenital posterolateral diaphragmatic hernia was made but on exploration there was a defect in the septum transversum along with features of Ivemark syndrome - asplenia with visceroatrial heterotaxia, malrotation and pancreatic divisum - an association not yet reported in literature. The child did well after operative correction of the hernia. Echocardiography showed situs inversus with dextrocardia with double outlet right ventricle, atrial septal defect, ventricular septal defect, patent ductus arteriosus and pulmonary stenosis.     

Surgical experience with congenital heart disease in Down's syndrome

Children with Down's syndrome and congenital heart defects have multiple problems. The role of cardiac surgery in the management of these patients was investigated by reviewing the clinical data, hospital course and follow-up of 21 patients (9 males and 12 females, age range 1 month to 14 years) with Down's syndrome and congenital heart defects operated in our institute. Twelve (57%) of these were infants and nine (43%), older children. Five were in congestive cardiac failure, four were hypothyroid. The heart lesions ranked in incidence as follows: atrioventricular septal defect 7 (33.3%), tetralogy of Fallot 3 (14.3%), tetralogy of Fallot & atrioventricular septal defect both 2 (9.5%), double outlet right ventricle with pulmonary stenosis 1 (4.8%), patent ductus arteriosus 2 (9.5%), patent ductus arteriosus plus coarctation 1 (4.8%), ventricular septal defect 2 (9.5%), atrial septal defect plus ventricular septal defect 1 (4.8%), atrial septal defect plus patent ductus arteriosus plus right pulmonary artery stenosis 1 (4.8%) and transposition of great arteries with multiple ventricular septal defect 1 (4.8%). Four (19%) patients had palliative procedures while the rest (81%) underwent primary repair. All survived the operation. The post-operative period was complicated in 6 (28.5%), with respiratory infections in 3, pulmonary hypertensive crisis in 2 and complete heart block in 1. The early mortality was 0, while there were 2 (9.5%) late deaths. The number of hospitalisations was markedly reduced according to the parents. Follow-up showed near normal pulmonary artery pressure in 50 percent children with large shunts and a good developmental spurt was seen in 60 percent. From a purely surgical viewpoint, the prognosis for children with Down's syndrome and congenital heart disease is good.