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PubMed2002

[Extracardiac fontan procedure in adult: report of a case].

We report a case of a 28-year-old female who underwent an extracardiac Fontan procedure. The subject was diagnosed as an atrioventricular septal defect (Rastelli classification: type C), a double outlet right ventricle, pulmonary artery stenosis, a hypoplasty of left ventricle, total anomalous venous return (Darling: Ib + IIb), and atrial flutter. She underwent a Blalock shunt and an aorto-pulmonary shunt at the ages of 3 and 9 years, respectively. Under a total CPB, an extracardiac total cavo-pulmonary connection (TCPC), using a 26 mm Hemashield graft, was completed. The postoperative course was uneventful. The complicated atrial anatomy and atrial arrhythmia indicated TCPC in this adult patient.

Adult↗
PubMed2003

Will a handheld ultrasound scanner be applicable for screening for heart abnormalities in newborns and children?

BACKGROUND: There is significant interest in opportunities to provide echocardiography services for detection of congenital heart disease with portable, or even handheld, devices in remote areas or third world countries where conventional ultrasound systems may not be available. We tested a handheld system (HHS) (SonoHeart, SonoSite Inc, Bothell, Wash) equipped with a broadband, 7- to 4-MHz, miniaturized, curved, linear-array transducer and implemented with an improved directional Doppler flow map. METHODS: All echocardiography scanning was performed in the neonatal nursery, pediatric intensive care department, or pediatric echocardiography laboratory of our institution. We reviewed limited echocardiography view sequences sequentially obtained by the same expert examiner (D.J.S.) in 50 infants and children (age: 1 day to 6 years), with preoperative or postoperative forms of congenital heart disease. Each patient was studied twice, once with a conventional full-feature system (FFS) and then a limited scan with the HHS using similar frequency transducers. The cardiologist (D.J.S.) and blinded research laboratory reviewers (X.L., G.K.M., R.A.R.) read the FFS and HHS image sequences for diagnosis and for grading the quality of the anatomic and flow feature images. The studies were performed and reviewed with the examiner and reviewers blinded to patient diagnosis. RESULTS: The major diagnoses (eg, patent ductus arteriosus, atrio-ventricular (AV) canal, peripheral pulmonary valve stenosis, aortic coarctation, atrial septal defect, ventricular septal defect, preoperative or postoperative tetralogy of Fallot, and mitral regurgitation) were made by both readers, who were unaware of each other's diagnosis results. Furthermore, the average composite HHS cardiac anatomic feature score on a scale of 0 (not visualized) to 3 (visualized precisely) from the parasternal long-axis and 4- or 5-chamber view for cardiac anatomy were 2.67 +/- 0.49 (SD) and 2.50 +/- 0.55, respectively, versus 2.73 +/- 0.45 and 2.55 +/- 0.54 for the FFS. The mean flow feature score, comprising all views, was 2.67 +/- 0.45 (HHS) versus 2.72 +/- 0.48 (FFS). The P values for all above comparisons were >.05. Image quality of the FFS anatomic structures were, thus, not statistically different from the HHS. Although the color cosmetic was different for the HHS directional (nonvelocity) map, only 9% of 150 total findings (including structural abnormalities and flow features, none of which were critical) were missed, whereas the other 91% regurgitant, shunt, stenosis flow features or heart structure were imaged adequately by the HHS in this population. CONCLUSIONS: Implementing high-frequency transducers and programs optimized for tissue and flow imaging on the HHS should provide images of sufficient quality for targeted echocardiography examinations to determine the presence, absence, or status of congenital heart disease in newborns and young children.

Aortic Valve↗
PubMed1987

[Percutaneous transluminal angioplasty in complex congenital cardiopathies. Apropos of 2 cases].

Percutaneous transluminal angioplasty (PTA), already widely used in stenosis of peripheral, renal or coronary arteries, has now been extended to congenital heart diseases. Thus, in pulmonary or aortic orificial stenosis this simple and fairly safe method has proved to be a suitable alternative to surgery. In other cases it may be used for tiding the patient over a critical period pending surgery. Finally, PTA may be performed as a palliative treatment of lesions that are too complex for surgery or carry an excessively high operative risk. This applies to the two cases reported here, where PTA enabled us to dilate: the ductus arteriosus in a patient with single ventricle--an application which, to our knowledge, has not yet been reported--and a strongly stenotic pulmonary orifice associated with a complex heart disease. In both cases PTA was successful both haemodynamically and angiographically and was followed by marked clinical improvement. The published cases of complex congenital heart diseases where PTA has been used are reviewed.

Adult↗
PubMed1997

Cardiopulmonary bypass venous cannulation challenges in a paediatric patient with complex congenital heart disease: a case report.

When choosing cannulae for cardiac surgery the two most important factors to be considered are the proposed procedure and the patient anatomy. These factors are especially crucial in paediatric patients with congenital heart disease. A 3-year-old, 14-kg male presented to the University of Iowa Hospitals and Clinics with dextro-transposition of the great arteries, atrioventricular canal, left pulmonary stenosis, azygous continuation, bilateral superior vena cavae, partial anomalous pulmonary venous return, left aortic arch and status post-right Blalock-Taussing shunt. The complex anatomy presented a surgical dilemma. The course of surgical intervention was determined, a variation of the modified Fontan procedure, and the anatomy of the patient was directly viewed. The surgeon concluded that four venous cannulae were required to provide adequate venous return for the cardiopulmonary bypass (CPB) circuit and a bloodless surgical field. The operation was successfully performed under mild hypothermia with no complications. The patient fully recovered with only mild restrictions on his activity level. This case acutely illustrates the importance of anatomical and procedural awareness when choosing cannulae and cannulation sites for CPB in paediatric patients with congenital heart disease.

Abnormalities, Multiple↗
PubMed2001

Cardiopulmonary malformations in the inv/inv mouse.

The inv/inv mouse carries an insertional mutation in the inversin gene, (inv, for inversion of embryonic turning). Previously it had been reported that almost 100% of the homozygous offspring (inv/inv) were characterized by situs inversus totalis. In this report we identify the spectrum of cardiopulmonary anatomical abnormalities in inv/inv mice surviving to birth to determine whether the abnormalities seen are of the categories classically associated with human situs abnormalities. Stillborn mice, offspring that died unexpectedly (within 48 hr after birth), and neonates with phenotypic characteristics of situs inversus (right-sided stomachs, growth failure or jaundice) were processed for standard histological examination. Of 173 offspring, 34 (20%) neonates (11 stillborn, 9 unexpected deaths, and 14 mice with situs inversus phenotype) were examined, 27 of which were genotyped to be inv/inv. Interestingly, three inv/inv mice (11%) were found to have situs solitus. Twenty-four had situs inversus with normal, mirror-image cardiac anatomy (dextrocardia with atrioventricular concordance, ventriculoarterial concordance and a right aortic arch). The overall incidence of cardiovascular anomalies observed was 10 out of 27 (37%). The most frequent severe malformation, identified in 3 out of 27 animals, was a complex consisting of pulmonary infundibular stenosis/atresia with absence of pulmonary valve tissue and a ventricular septal defect. The pulmonary phenotype in inv/inv mice was situs inversus with occasional minor lobar abnormalities. We conclude that 1) cardiopulmonary malformations in inv/inv mice are not rare (37%), 2) the cardiopulmonary malformations observed in inv/inv specimens are not of the spectrum typically associated with human heterotaxia. In particular, inv/inv mice have a propensity for defects in the development of the right ventricular outflow tract and the interventricular septum, and 3) approximately one out of ten inv/inv mice is born with situs solitus and shows cardiac anomalies that correspond to those observed in inv/inv specimens with situs inversus. Our data therefore suggest that inversin, the product of the inv locus, may have specific roles in cardiac morphogenesis independent of its role in situs determination.

Animals↗
PubMed2003

[Early complications of stenting in children and young adults with congenital heart disease].

We report the early complications of stent implantation. Since 1/1/97 to 1/12/02, we implanted 91 stents in 76 patients. Median age and weight were 15 years (range 0.3-43) and 49 kg (range 3-96). Primary diagnosis were: pulmonary atresia with ventricular septal defect (n = 17), tetralogy of Fallot (n = 13), aortic coarctation (n = 15), transposition of the great arteries (n = 3), truncus arteriosus (n = 6), univentricular heart (n = 7), other anomalies (n = 28). Indications to stent implantation were: stenosis of the pulmonary outflow and/or pulmonary branches (n = 44), aortic recoarctation (n = 14), native aortic coarctation (n = 1), systemic venous channels obstruction (n = 14), other (n = 3). The stents were: Palmaz (n = 57), non premounted CP (n = 8), premounted CP (n = 3), Palmaz Corinthian (n = 19), self-expanding (n = 4). Procedural complications occurred in 24 patients (31%) and included: 11 balloon bursting, 8 stent slipping, 1 stent migration, 4 wall dissections. Five patients (21%) needed urgent surgery. Sixty three percent of complications occurred during pulmonary outflow or pulmonary branches stenting, 25% during stenting of the aortic arch, 12% during other procedures. All but 2 complications were observed with non premounted stents. In conclusion, the use of not premounted stents, although widely diffuse, has a not negligible incidence of complications, leading to longer procedures, to the use of a large variety of materials and needing more than occasionally a surgical solution. A larger use of premounted stents, when possible, is recommended.

Adolescent↗
PubMed1994

[Cardiopulmonary capacity of patients with congenital heart defects in childhood, adolescence and adulthood].

Cardiopulmonary exercise capacity is a significant criterion of life quality. The evaluation of the exercise capacity is important to answer patient-questions concerning every day activity, choice of profession, sports-activity etc. We performed cardiopulmonary exercise testing in 38 patients (age 33.6 +/- 12.0 years, 18 women, 20 men) with different congenital heart disease (5 after surgical repair of tetralogy of fallot, 2 after Mustard-operation in transposition of the great arteries (TGA), 1 single ventricle, 14 atrial septal defect (ASD), 8 ventricular septal defect (VSD), 8 pulmonary valve stenosis (PS)) during outpatient routine control. All tests were performed on upright bicycle with continuous ramp program of 20 Watt increase/minute. Ventilatory values as O2-uptake, CO2-production, minute ventilation (VE) were measured breath-by-breath. Max. VO2 was reduced as average value for every patient group (tetralogy of fallot 60.2 +/- 20.3% pred., TGA 53.0 +/- 0.0% pred., single ventricle 35% pred., closed ASD 71.9 +/- 23.8% pred., ASD 62.7 +/- 30.0% pred., VSD 64.1 +/- 11.7% pred., PS 73.2 +/- 16.0% pred.). Anaerobic threshold was reduced in tetralogy of fallot (35.9 +/- 12.2% pred. max. VO2) and in single ventricle (28.3% pred. max. VO2). In comparison with clinical classification of exercise capacity we found for max. VO2 differences in 23/38 patients. 22/23 patients reported no exercise limitation but had reduced max. VO2. One patient had a normal max. VO 2 but complaints of exercise dyspnoea. For anaerobic threshold 18/38 patients had discrepancies in objective and subjective estimation of their exercise capacity.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1987

[Univentricular heart and pulmonary stenosis with a right-to-left shunt between the innominate and pulmonary veins after Glenn operation: a case report].

A 12-year-old girl with a univentricular heart and pulmonary stenosis, who had undergone Glenn operation at two years of age and Fontan operation at 11 years of age, had a right-to-left shunt via collaterals between the innominate and pulmonary veins. This right-to-left shunt was detected by two-dimensional contrast echocardiography. The contrast material was injected into the left antecubital vein appeared in the left atrium, and this was confirmed by innominate venography. The contrast material injected into the left innominate vein passed via the collaterals into the pulmonary veins bilaterally. No such cases have so far been reported. In our experience, in four of six cases after Glenn operation, including the present case, the abnormal collateral circulation from the superior vena cava to the atrium was detected by two-dimensional contrast echocardiography. This case is interesting embryologically and suggests that the connection between the pulmonary and systemic veins which is present in early fetal life, and subsequently disappears, was present after birth.

Adolescent↗
PubMed1981

Long-term results of operated and non-operated patients with congenital heart diseases.

The long-term results of 1023 adult patients with congenital heart diseases, operated and non-operated, with atrial septal defect (ASD), patent ductus arteriosus (PDA), pulmonary stenosis (PS) and tetralogy of Fallot, were followed for up to 24 years, and the long-term results of operated and non-operated congenital heart diseases were compared. The survival rate of patients with operated isolated ASD was significantly higher than in patients with non-operated isolated ASD, and that of operated patients with VSD associated with other anomalies was also significantly higher than in non-operated VSD patients with such involvement. The survival rate showed no significant difference in the operated and non-operated patients with PDA, PS and tetralogy of Fallot. In all congenital heart diseases, there was a close correlation between the mean pulmonary artery pressure and the mortality rate both in operated and non-operated patients and a marked improvement of physical capacity of operated patients at the time of the last follow-up examination.

Adolescent↗
PubMed1979

Hypertensive pulmonary vascular disease in Down syndrome.

Clinical studies have suggested that patients with Down syndrome have precocious development of pulmonary hypertension, even in the absence of congenital heart disease. To examine the pathologic basis of this impression, we studied 82 patients with Down syndrome autopsied at The Johns Hopkins Hospital from 1913 to present. The patients ranged in age from 26 hours to 25 years; 41 (50%) were female. Atrioventricular canal defect was present in 40 patients, nine of whom also had pulmonary stenosis. Eight had isolated ventricular septal defect and seven had other malformations. Histologic sections of the lung from each patient were studied and the degree of hypertensive pulmonary vascular disease graded. Age and sex-matched controls without cardiovascular manifestations, all other autopsied patients with atrioventricular canal defect not associated with Down syndrome, and age-matched patients with ventricular septal defect or other malformations were similarly studied for pulmonary vascular changes. Comparison of the 27 patients with Down syndrome and no cardiovascular malformations with normal controls showed no differences, with one notable exception: an 8-month-old child with Down syndrome had severe HPVD secondary to idiopathic pulmonary hypertension. HPVD in patients with the various cardiovascular malformations was similar for those groups with Down syndrome and those without, when HPVD was considered as a function of the patients' age and the type of malformation. We conclude that, in general, patients with Down syndrome have no predisposition to develop severe or precocious hypertensive pulmonary vascular disease.

Adolescent↗
PubMedDate not supplied

[Management of pulmonary stenosis in the correction of the tetralogy of Fallot].

Successful surgical correction of (la estenosis pulmonar en la tetralogía de Fallot), requires that the postoperative ratio between the (right ventricular pressure) and (left ventricular pressure) (PVD/PVI) be less than 0.70. By measuring the ring diameter and pulmonary branch on relation to the (superficie corporal) it is possible to predict the outcome of this ratio and therefore indicate whether or not it is necessary to increase (AP). This paper presents our experience with 24 patients subjected to total correction of (tetralogía de Fallot) from March of 1975 to the present using the above criteria. The average age was 7 years (ranging from 2 to 20). In all cases the (cresta supraventricular) was (resected). In 14 (58%) a (valvultomia pulmonar) was performed and in 9 patients (37) it was necessary to increase the ring and pulmonary branch of the pulmonary artery according to measurements taken. The post-operative relationship PVD/PVI was 0.2-0.3: 15%, 0.4-0.5: 60%, 0.6-0.7: 25% In no case was the ratio greater than 0.70. No significant difference was found between those cases requiring... (parche transanular) and those where such treatment was not necessary. Three patients (12%) died immediately following the operation. It is concluded that measurement of the diameter of the pulmonary ring in comparison to the (superficie corporal) is extremely useful in management during surgery of (estenosis pulmonar) in patients with (tetralogía de Fallot).

Humans↗
PubMed1983

[Mitral valve prolapse in the heart with pressure overload of the right ventricle].

The prevalence of mitral valve prolapse (MVP) was examined in 16 patients with right ventricular pressure overload with a peak systolic pressure ranged from 60 to 204 mmHg (Group 1; 9 cases of primary pulmonary hypertension and 7 cases of pulmonary valve stenosis), and the clinical and echocardiographic data were compared to those of 12 patients with idiopathic MVP (Group 2) and 10 healthy persons (Group 3). MVP was defined when either or both of the mitral valve leaflets protruded into the left atrium beyond the plane of the mitral ring, and "gap" was defined as positive when one of the leaflets of the mitral valve slipped 3 mm or more without prolapse toward the left atrium than the other leaflet. Eccentricity of the left ventricle was obtained from the cross-sectional view of the left ventricle at the mid-ventricular level: the ratio of the internal diameter (S) passing through the mid-point of the interventricular septum (IVS) and perpendicular to the IVS divided by the longest internal diameter (L) parallel to the IVS, at end-diastole (S/L(d] and at end-systole (S/L(s], respectively. The following results were obtained. MVP was found in seven cases of 16 patients (43.8%) of Group 1. Two cases had positive "gap" (12.5%). Eccentricity of the left ventricle in Group 1 (S/L(d) 0.81 +/- 0.12, S/L(s) 0.78 +/- 0.15) was more marked than that of Group 2 (S/L(d) 0.98 +/- 0.09, S/L(s) 1.02 +/- 0.06) or of Group 3 (S/L(d) 1.03 +/- 0.08, S/L(s) 1.00 +/- 0.04) (p less than 0.001 in each).(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1995

Experience with repair of congenital heart defects using adjunctive endovascular devices.

The use of endovascular devices as an adjunct to repair of congenital heart anomalies represents a novel but unproven therapeutic approach. Intraoperative implantation of pulmonary arterial stents (5 to 15 mm diameter) was done in 11 patients with pulmonary atresia with ventricular septal defect (n = 4), classic tetralogy of Fallot (n = 2), truncus arteriosus (n = 1), hypoplastic left heart syndrome (stage II [n = 1] and stage III [n = 1] Norwood procedure), and miscellaneous pulmonary arterial stenoses (n = 3), as well as in patients with congenital (n = 1) and postoperative (n = 3) pulmonary venous obstruction and in 1 patient with combined pulmonary arterial and venous obstruction. The stents were effective at achieving immediate patency in all patients. There were two early deaths, one related to acute thrombosis of a small-diameter left pulmonary artery stent. Reintervention because of stent-related pulmonary arterial stenosis was frequently necessary. In five of seven patients who survived more than 1 month after implantation of stent size 8 mm or smaller severe stent-related pulmonary arterial obstruction developed. In four of the five patients with pulmonary vein stent implantation intractable obstruction developed, resulting in death in all three patients who had bilateral pulmonary vein stent implantation. Intraoperative occlusion of apical muscular ventricular septal defect with use of a clamshell device inserted from the right atrial approach was accomplished in four patients. One patient who underwent associated aortic arch reconstruction died as a result of left ventricular hypoplasia. The results in the remaining three patients were favorable on the basis of absence of significant late residual intraventricular shunting, left ventricular dysfunction, or arrhythmia. We conclude that recurrent intraluminal obstruction as a result of neointimal hyperplasia appears to be an eventual certainty in currently designed small-diameter endovascular stents. For this reason, we would recommend standard surgical techniques for repair of obstructive lesions of the pulmonary arterial confluence to maximize growth potential. Device occlusion of muscular ventricular septal defects is feasible but probably only indicated for complex cases of ventricular septal deficiency that otherwise necessitate a left ventriculotomy.

Adolescent↗
PubMed1993

Management of typical and dysplastic pulmonic stenosis, uncomplicated or associated with complex intracardiac defects, in juveniles and adults: use of percutaneous balloon pulmonary valvuloplasty with eight-month hemodynamic follow-up.

To alleviate large fixed right ventricular (RV) outflow gradients, percutaneous balloon dilatation of pulmonic stenosis (PS) was performed in 38 patients with mean age of 14 +/- 14 years (median: 9 years, age range: 9 months to 63 years). There were 21 males and 17 females. Thirty-four patients had typical PS (5 of them also having other complex congenital cardiac anomalies, while 13 additional patients had a patent foramen ovale); 2 further subjects had subpulmonic, and 2 dysplastic pulmonary valvular obstructions. Sixteen patients were in the New York Heart Association (NYHA) Class I, 15 in Class II, 6 in Class III, and 1 in Class IV. Electrocardiographic (ECG) evidence of right ventricular hypertrophy (RVH) was present in 29 patients (76%); 3 patients had right bundle branch block (RBBB). For the entire group, there was a marked decrease in the mean systolic transpulmonic gradient in the immediate post-valvuloplasty period (from 97 +/- 43 to 26 +/- 17 mmHg; P < 0.0001). One patient expired 8 hours post-valvuloplasty (he was in the NYHA Class IV, and had severe RV failure). No other cardiovascular complications were encountered; the median hospital stay was 3 days (range: 1-10 days). At an 8-month follow-up, 12 patients who were reevaluated invasively had a median transpulmonic gradient of 27 mmHg (range: 5-92 mmHg) as compared to their pre-valvuloplasty values of 84 mmHg (range: 49-142 mmHg; P < 0.004).(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed2006

Clinical implications of major aortopulmonary collateral arteries in patients with right isomerism.

BACKGROUND: The presence of major aortopulmonary collateral arteries (MAPCA) in patients with right isomerism may alter the prognosis, which is still unclear. METHODS: From 1994 to 2003, 138 out of 155 patients (89%) with right isomerism had pulmonary stenosis or pulmonary atresia and constituted the study population. RESULTS: Two groups of patients were identified, one with MAPCA and the other without. The MAPCA originating from descending thoracic aorta or aortic arch was noted in 9 patients (6.5%), with a median number of MAPCA being 3 (range, 2 to 4). Patients with MAPCA commonly had congestive heart failure (8/9, 89%) and frequent lower airway infection (6/9, 67%). In comparison with patients with MAPCA and without, the intrapericardial pulmonary artery was more frequently absent (2 of 9 vs 1 of 129, p < 0.001), and the pulmonary arterial size was smaller (McGoon ratio <1.5, 8 of 9 vs 57 of 129, p = 0.009) in the MAPCA group. The mean pulmonary arterial pressure measured was often higher than the suggested limit for Fontan circulation. Major aortopulmonary collateral arteries were addressed at a median of 6 months old (2 to 19 months old) in 5 patients. Although surgical mortality was not different for first-stage palliation and Glenn shunt in both groups, total cavopulmonary connection could only be performed in one patient with MAPCA with poor results. However, the 5-year survival of patients with and without MAPCA was not statistically different (74.1% vs 55.2%, p = 0.19). CONCLUSIONS: The presence of MAPCA was uncommon but still noted in patients with right isomerism. It may cause heart failure and a less favorable pulmonary vasculature for a complete Fontan-type operation.

Abnormalities, Multiple↗
PubMed2003

Subcortical band heterotopia with simplified gyral pattern and syndactyly.

We describe a girl with an unusual form of subcortical band heterotopia (SBH) and a complex malformation syndrome. SBH had an irregular inner margin, organized in contiguous fascicles of migrating neurons, sometimes giving the appearance of many small contiguous gyri. The true cortex had decreased thickness and showed a simplified gyral pattern with decreased number of gyri, which were usually of increased width, and shallow sulci. The cerebellum was hypoplastic. Additional features included epicanthal folds, hypertelorism, small nose with hypoplastic nares, bilateral syndactyly of the toes, pulmonary valve stenosis, atrial and ventricular septal defects. At the age of 1 year the patient had severe developmental delay and epilepsy. Chromosome studies and mutation analysis of the DCX and LIS1 genes gave negative results. This observation delineates a new multiple congenital abnormalities mental retardation syndrome and confirms genetic heterogeneity of SBH.

Brain↗