bluuebunny/crossref_metadata_2025 huggingface dataset:
Changes in patterns of left ventricular diastolic filling revealed by Doppler echocardiography in infants with ventricular septal defect
Authors: Ito Tadahiko , Harada Kenji , Tamura Masamichi , Takada Goro
Published: August 2008

To evaluate left ventricular diastolic filling in infants with ventricular septal defect, which has yet to be documented, we measured various Doppler echocardiographic indexes from transmitral flow in the following groups: 10 infants with ventricular septal defect without pulmonary hypertension; 10 infants with ventricular septal defect with pulmonary hypertension; and 9 normal infants to serve as controls. The peak A, total velocity time integral, E area, and A area in patients without pulmonary hypertension were all significantly larger than those in controls. The peak ratio E/A, and 1/3 filling fraction, in patients without pulmonary hypertension were significantly lower than in controls. The peak A, A area, and deceleration time in patients with pulmonary hypertension were significantly larger than in patients without pulmonary hypertension and controls. The peak E/A, area E/A, and 1/3 filling fraction in patients with pulmonary hypertension were significantly lower than in those without pulmonary hypertension and controls. The index of left ventricular mass, as well as the index of end-diastolic left ventricular wall thickness, correlated strongly with peak A, A area, and deceleration time. The ratio between the systolic pulmonary and systemic pressures correlated strongly with peak A, A area, peak E/A, area EtA, and 1/3 filling fraction.

These results demonstrated that the patterns of left ventricular filling in infants with ventricular septal defect were different from those in normal infants, and suggested that the abnormal patterns may indicate the insufficiency of adaptation of left ventricle (increase of left ventricular compliance) for volume overload in the presence of a ventricular septal defect..
Sternal defects associated with congenital pericardial and cardiac defects
Authors: Cottrill Carol M. , Tamaren Janet , Hall Brian
Published: August 2008

We describe three cases of sternal defects of varying severity associated with other congenital anomalies. In the most severe case, both anterior and posterior defects were seen, with near-absence of the sternum and pericardium continuous with a large omphalocele. This resulted in external location of organs usually confined within the thoracic and abdominal cavities. A ventricular septal defect was present, and the arterial duct was absent. The course of the ascending aorta was anomalous. The baby had anencephaly and rachischisis. In the intermediate case, a proximal sternal cleft was associated with shortening of the ster num, and absence of the manubrium. Anterior pericardial and diaphragmatic defects were seen, while a scalp defect and an encephalocele were present on the posterior aspect of the head. This baby had tricuspid atresia. The qremaining case had only an anterior defect with a shortened sternum. A supra-umbilical omphalocele contained a left ventricular diverticulum without interposing pericardium or diaphragm. Ventricular and atrial septal defects were present. The first two cases can be considered as representing fail ure of development of both an anterior and a posterior midline field. The third case, much milder than the other two, represents failure of development of an anterior field.
Repair of tetralogy of Fallot associated with atrioventricular septal defect
Authors: Tláskal Tomás̆ , Huc̆ín Bohumil , Kostelka Martin , Chaloupecký Václav , Marek Jan , Tax Petr , Janouàek Jan , Kuèera Vladimír , Hruda Jaroslav , Reich Oleg , S̆kovránek Jan
Published: August 2008

Tetralogy of Fallot, when associated with atrioventricular septal defect permitting shunting at ventricular level, represents a complex cyanotic congenital malformation. Experience with surgical repair is limited, and results vary considerably. Between 1984 and 1996, we repaired 14 consecutive patients with this combination seen in our center. Their ages ranged from 8 months to 21 years (median 7.4 years). Six (42.9%) had Down's syndrome. In eight patients the correct diagnosis was made using echocardiogra phy alone. In the remaining six patients, who had previously-constructed arterial shunts and/or suspected pulmonary arterial stenosis, catheterization and angiocardiography were also performed. The repair consisted of double patch closure of the septal defect, reconstruction of two atrioventricular orifices, and relief of pulmonary stenosis at all levels. In five patients with a hypoplastic pulmonary trunk, a monocusp transannular patch (four patients) or an allograft (one patient) was used for restroration of continuity from the right ventricle to the pulmonary arteries. Patch enlargement of one or both pulmonary arteries was necessary in five patients. One patient (7.1%) died early, and another late. The twelve surviving (85.8%) patients have been followed for 1.2–12.5 years after surgery (median 4.9 years, mean 5.9 ± 3.9 years). During the follow-up, reoperation was necessary for repair of residual ventricular septal defect and pulmonary regurgitation in two patients, and closure of an atrial septal defect and alteration to left atrioventricular valvar regurgitation in one patient. Seven patients are in class I of the New York Heart Association, four in class II, and one in class III. Tetralogy of Fallot associated with atrioventricular septal defect can be corrected with low mortality and good long-term results. Residual lesions, however, have a tendency to progress, especially when seen in combination. After surgery, all patients need long-term close follow-up.
Thiamine deficiency mimicking acute rejection following cardiac transplantation
Authors: Gennery Andrew R. , Bartlett Kim , Hasan Asif
Published: August 2008

We describe a recipient of an orthotopic cardiac transplant who developed severe ventricular dysfunction following an episode of sepsis. He had normal cardiac biopsies and responded to treatment with thiamine. Causes other than rejection should be considered in patients who have received a cardiac transplant and who present in cardiac failure.
Intracardiac thrombus associated with resistance to activated protein C
Authors: Kohlhase Bernd , Kececioglu Deniz , Nowak-Göttl Ulrike
Published: August 2008

Formation of thrombus in the heart is a rare event in children. Genetic risk factors determined for thrombophilia with additional exogenous risk factors may, none the less, promote cardiac thrombosis. We report on a boy without predisposing cardiac disease, heterozygous for resistance to activated protein C, who developed multiple thrombuses attached to the mitral valve which led to recurrent arterial throm boembolism. In addition this patient showed enhanced levels of lipoprotein (a).
Fetal ventricular tachycardia in long QT syndrome
Authors: Yamada Miho , Nakazawa Makoto , Momma Kazuo
Published: August 2008

We report a case of a fetus presenting intermittent ventricular tachycardia during midgestation, whose mother had long QT syndrome. The arrhythmia disappeared spontaneously, and the electrocardiogram of the baby after birth showed prolonged QTc. As far as we are aware, this is the first case in which ventricular tachycardia was documented during fetal life in a baby with long QT syndrome.
Surgical treatment for aortic regurgitation and left ventricular dysfunction caused by Kawasaki disease
Authors: Takahashi Toshiki , Taniguchi Kazuhiro , Matsuda Hikaru
Published: August 2008

A 10-year-old girl required replacement of the aortic valve because of massive regurgitation caused by Kawasaki disease at the age of 3 months. Postoperatively, left ventricular function and mass improved remarkably, with reduction of both hypertrophy and interstitial fibrosis. During a follow-up period of 10 years, she has been free from any cardiac event.
Unusual presentation during childhood of left ventricular myxoma
Authors: Kapoor A. , Radhakrishnan S. , Sinha N.
Published: August 2008

Amongst all primary cardiac tumors, myxomas are the commonest, and their commonest site of origin is the left atrium. Myxomas originating in the left ventricle are rare. When seen, they usually present with a history of systemic embolisation and/or syncopal episodes, with constitutional symptoms being absent. We report here a child with left ventricular myxoma who presented with a prolonged febrile illness.
Holt-Oram syndrome and multiple ventricular septal defects: an association suggesting a possible genetic marker?
Authors: Bennhagen Rolf G. , Menahem Samuel
Published: August 2008

A family is described where the father has the many skeletal, but none of the cardiac abnormalities associated with the Holt-Oram syndrome. His two daughters have similar skeletal anomalies, but with identical cardiac lesions, as does another patient, raising the possibility of an associated genetic marker.
Left isomerism with tetralogy of Fallot and anomalous systemic and pulmonary venous connections
Authors: Da Cruz E. , Milella L. , Corno A.
Published: August 2008

The association of left isomerism with interruption and azygos continuation of the inferior caval vein, anomalous connection of the right pulmonary veins to the right atrium, an interatrial commu nication of sinus venosus type, and tetralogy of Fallot with right aortic arch and anomalous coronary artery has been observed in a 6-month-old patient. As far as we are aware, the association of these congenital car diac malformations has not previously been reported in patients with isomeric left atrial appendages.
Surgical ablation of a cardiac rhabdomyoma in an infant with tuberous sclerosis
Authors: Henglein Dagmar , Guirgis Nabil-Maurice , Bloch Gérard
Published: August 2008

Cardiac rhabdomyoma is a rare tumor. Because of its rate of regression, mainly in the first year of life, conservative therapy is generally suggested when diagnosis occurs in infancy. We report surgical ablation of a rhabdomyoma in a 2-month-old boy in whom the tumor extended into the subaortic outflow tract. The tumor, which had a dual attachment to the aortic and mitral valves, was safely removed without any valvar damage. We suggest immediate surgical intervention for those rhabdomyomas which are life-threatening because of their size, location, or arrhythmogenicity.
Rheumatic fever
Authors: Cleonice de Carvalho Coelho Mota  , Meira Zilda Maria Alves
Published: August 2008

In recent years, rheumatic fever has been declining in the industrialized countries, and has became a rare disease. In developing areas, nonetheless, the sequels of its cardiac involvement have important implications from the stance of public health, and this disease is still the main cause of heart disease in children and young adults. From a historical perspective, the long-term prevention and the control of socioeconomic problems have been proven to be effective, and it is these measures which have contributed to the decline of the disease in developed countries. But, due to its present high prevalence around the world, and the potential for his resurgence, this disease remains an unsolved problem. Furthermore, the complete understanding of its pathophysiology remains a challenge. In this presentation, we will discuss our experience with epidemiological, clinical and prophylatic aspects of this enigmatic disease, and the strategies we have developed to study and control heumatic fever in Minas Gerais, Brazil.
Complex pulmonary atresia in an adult: natural history, unusual pathology and mode of death
Authors: Thierrien J. , Gerlis L. M. , Kilner Philip , Somerville Jane
Published: August 2008

A patient with unrepaired complex pulmonary atresia had a normal life, achieving two successful pregnancies, until the age of 44 years. Confluent central pulmonary arteries were supplied by a fistu-lous communication from the left coronary artery, and from other collateral arteries arising from the underside of the aortic arch. Unusual aneurysms were present. Death at the age of 46 resulted from dissection and rupture of an aneurysmal dilation of the pulmonary trunk.
Experience with the Glenn anastomosis in the adult with cyanotic congenital heart disease
Authors: Elizari Amalia , Somerville Jane
Published: August 2008

A clinical study on the outcomes of Glenn anastomoses performed since 1987 in eight consecutive patients aged ≥16 years, and in two performed earlier, showed poor results. One badly selected patient died early as a consequence of high venous pressure, while a further seven had early complications. Seven of eight hospital survivors were followed for 1–10 (median 4.2) years with two deaths (1 and 4 years later). Of the remaining five patients, two improved temporarily, but increased arterial oxygen saturation was not maintained after 6 months. The two patients who had undergone a Glenn anastomosis 10 and 34 years earlier were shown to have pulmonary arteriovenous fistulas. The Glenn anastomosis in these older patients is associated with high rates of complication and appears not to give adequate palliation, particularly when it is the only source of pulmonary blood supply. In the adult, the Glenn anastomosis can be used as a staging procedure for Fontan-type surgery, but must be combined with another source of pulmonary arterial supply. Any adult having a Glenn anastomosis, particularly without another source of pulmonary arterial supply, should be warned of the possibility of worsening of cyanosis and symptoms. The second stage of the procedure may need to be performed soon after the first should the hypoxia prove intolerable.
Acute complications in the current era of therapeutic cardiac catheterization for congenital heart disease
Authors: Zeevi Benjamin , Berant Michael , Fogelman Rami , Galit Bar-Mor , Blieden Leonard C.
Published: August 2008

The acute complications of therapeutic cardiac catheterization for congenital heart disease as performed currently in a small unit were reviewed. In recent years, there has been a significant increase in the number of lesions thought amenable to catheter therapy. Only a few reports, however, have addressed the overall incidence of acute complications of therapeutic cardiac catheterization, all representing the experience of centres performing moderate-to-large numbers of procedures. A retrospective review was performed of 425 therapeutic catheter procedures performed at our institution between May 1993 and November 1997. Acute complications were retrieved from the database. This included all adverse events that were clinically recognized at the time of or within 2 weeks after the procedure and which, to the best of the authors' clinical judgement, were related to the catheterization and not part of the natural history of the child's illness. All patients were observed overnight following the procedure, and stayed in hospital if a complication developed. There were 49 acute complications (11.5%), of which 43 (10.1%) were deemed minor and 6 (1.4%) were considered major. The rate was low in patients with valvar pulmonary stenosis, including three neonates (3/45, 6.7%), for those undergoing angioplasty of native co-arctation (1/15, 6.7%) and pulmonary arteries (2/21, 7.4%); and for coil embolization of systemic to pulmonary collateral arteries (1/16, 6.3%). The rate was high in patients with valvar aortic stenosis, including 12 neonates (9/37, 24.3%), and for angioplasty of re-coarctation (4/23, 21.7%). There were more overall complications in neonates (25.6%) than in older patients (10.1%) (p< 0.01). Two patients died (0.5%), but no patient required emergency surgical intervention. In spite of the introduction of many new therapeutic modalities with greater intrinsic risk, and the fact that patients with more complex lesions and who are more acutely ill are being treated, the overall rate of complications remains relatively low. This probably reflects improvements in pericatheterization medical management, in selection of patients, in procedural techniques, and in the experience of operators.
Tachycardias in children originating in the right ventricular outflow tract: lack of clinical features predicting the presence and severity of the histopathological substrate
Authors: Drago Fabrizio , Mazza Andrea , Gagliardi Maria Giulia , Bevilacqua Maurizio , Renzi Paolo Di , Calzolari Armando , Francalanci Paola , Boldrini Renata , Bosman Cesare , Liso Gaetano Di , Ragonese Pietro
Published: August 2008

The aim was to determine whether the clinical features of tachycardias originating from the right ventricular outflow tract in children with an apparently normal heart could predict the presence and the severity of the histopathological substrate. Thirteen children (median age 6 years; range 6 months–12 years) with tachycardia originating from the right ventricular outflow tract of apparently normal hearts, were assessed by echocardiography, heart catheterization with angiography, endomyocardial biopsy (13 patients) and magnetic resonance imaging (MRJ) (nine patients). Tachycardia was symptomatic in six and sustained in nine. Endomyocardial biopsy and MRI revealed acute myocarditis in five patients (38%), fatty infiltration of the right ventricle in two (15%), and minor histologic abnormalities in three (23%). Myocarditis was diagnosed in three of nine patients with sustained ventricular tachycardia, as opposed to two of four with non-sustained tachycardia (p = NS); in three of six symptomatic versus two of seven asymptomatic patients (p = NS); and in two of eight patients in whom ventricular tachycardia was induced during exercise testing as opposed to one of three in which it was not inducible (p = NS). A histopathological substrate was found in six of nine patients with sustained ventricular tachycardia, and in all four with non-sustained tachycardia (p = NS); in five of six patients with symptoms versus five of seven asymptomatic patients (p = NS); and in five of eight with inducible ventricular tachycardia during exercise testing versus all three in whom it was not inducible (p = NS). The mean rate of tachycardia was 184 ± 39 beats min−1 in patients with myocarditis, as opposed to 171 ± 48 in patients without myocarditis (p = NS); and 163 ± 33 in patients with a histopathological substrate compared with 210 ± 65 in patients without a histopathological substrate (p = NS). It is concluded that a histopathological substrate is present in the greater majority of children affected by the so-called right ventricular outflow tract tachycardia, but that the clinical features of the tachycardia do not predict the presence and the severity of this histopathological substrate.
Sternal wound and mediastinal infections in infants with congenital heart disease
Authors: Kearns Barbara , Sabella Camille , Mee Roger B. B. , Moodie Douglas S. , Goldfarb Johanna
Published: August 2008

The objective was to describe the epidemiologic, clinical, bacteriologic and therapeutic features of seven infants who developed sternal wound and mediastinal infections following palliation and/or repair procedures for congenital heart disease. A retrospective chart review was used. All infants with sternal wound and mediastinal infections were < 30 days of age at the initial operative procedure. Six of the infants had hypoplastic left heart syndrome, and one had complete transposition. Two infants required delayed closure of their chest wound. Three infants had superficial sternal infections and presented at a mean of 12 days postoperatively. Four infants had infection of the deep mediastinal structures: they were all asymptomatic and had purulent collections in their mediastinum at their second palliative operation, which was performed at a mean of 120 days after the initial surgery.Staphylococcus aureus, or coagulase-negativeStaphylococcus, was isolated from the wound and/or blood of six infants. All infants with mediastinal infections were managed with operative debridement. Infants with superficial infections underwent local debridement. All infants received long-term intravenous antibiotics. Mediastinal infections in infants undergoing palliative staged procedures for congenital heart lesions may be chronic and indolent, resulting in delayed repair of congenital heart lesions.
Persistence of the left superior caval vein: can it potentiate obstructive lesions of the left ventricle?
Authors: Agnoletti Gabriella , Annecchino Francesco , Preda Laura , Borghi Adele
Published: August 2008

Recent evidence has suggested that persistence of the left superior caval vein is associated with a high incidence of obstructive lesions of the left heart. To shed more light on this issue 1085 patients with congenital heart disease were studied retrospectively, with the aim of estimating the prevalence of a persistent left superior caval vein and its associated anomalies, focusing attention on obstructive lesions in the left and right ventricles. Patients with isomerism of the atrial appendages, or hypoplastic left heart syndrome, were excluded. A persisting left superior caval vein was present in 57 patients (5.2%). The overall incidence of obstructive lesions of the left heart was higher in patients with than in those without a persistent left superior caval vein (31.6 versus 7.8%,p< 0.001). Relative hypoplasia of the left ventricle was also higher in patients with persistent left superior caval vein (14 versus 0.8%,p< 0.001). The obstructive lesions found in the left heart, compared with the number in those without a left caval vein, were: mitral stenosis, 5.2 versus 0.7%; subaortic stenosis, 5.3 versus 0.9%; aortic coarctation, 17.5 versus 5.8% (p< 0.01); all of these in association, 3.5 versus 0.4%. In contrast, the incidence of obstructive lesions of the right heart was similar in the two groups of patients. It is concluded that persistence of the left superior caval vein can perturb the normal development of the left ventricle, being strongly associated with obstructions to left ventricular inflow and outflow.
The Italian Multicentric Study on Epidemiology of Congenital Heart Disease: first step of the analysis
Authors: Bosi Giuliano , Scorrano Marco , Tosato Giovanna , Forini Elena , Chakrokh Roxana ,   and the Working Party of the Italian Society of Pediatric Cardiology
Published: August 2008

We present the aims, methodology and initial results from the Italian Multicentric Study for the registration and follow-up of congenital heart disease. The general aims are to measure the prevalence of congenital heart disease in different geographic areas of Italy, and to assess the survival and outcome of affected babies. During the years 1992 and 1993, eighteen centers for Pediatric Cardiology spread all over the Country enrolled 1445 new babies with congenital cardiac malformations from a population of 341, 647 surveyed livebirths. The new cases were registered using the same methodologic criterions of the EUROCAT study in order to evaluate differences and/or similarities between the studies. The prevalence varied between 1.8‰ and 8.1‰; the average being 4.6%. The large range in prevalence is presumed to be related to different customs and hierarchies in flow and referral of patients. We provide total prevalence of individual lesions, and distribution of sentinel cardiac anomalies, in the Italian study and compare them with EUROCAT. Isolated ventricular septal defect is the most common lesion (39%); followed by atrial septal defect (7.5%); pulmonary valvar stenosis (7.3%); atrioventricular septal defects (5.4%); patency of the arterial duct (3.8%); complete transposition (3.7%); tetralogy of Fallot (3.3%); aortic coarctation (2.4%); aortic valvar stenosis (2.2%); and left heart hypoplasia (1.8%). The echographic stratification of ventricular and atrial septal defects, by location and size, was in keeping with the findings of the EUROCAT study. Because of the recent widespread availability of color-Doppler tecniques, the stratification of aortic and pulmonary valvar stenosis was an innovative approach in our study. Among the complex cardiovascular anomalies, double inlet ventricle and pulmonary atresia had a proportion of about 2% each; with double outlet right ventricle, common arterial trunk, Ebstein's malformation, tricuspid atresia, interrupted aortic arch and totally anomalous pulmonary venous connection having a proportion ranging from 0.5 to 0.8%. We discuss clinical features, such as frequency of extracardiac anomalies and familial aggregation of congenital heart disease, in comparison with the EUROCAT data.
Asymmetrically short tendinous cords causing congenital tricuspid regurgitation: improved understanding of tricuspid valvar dysplasia in the era of color flow echocardiography
Authors: McElhinney Doff B. , Silverman Norman H. , Brook Michael M. , Hanley Frank L. , Stanger Paul
Published: August 2008

Background

Tricuspid regurgitation as a manifestation of an isolated congenital anomaly of the tricuspid valve is rare. Cross-sectional and color Doppler echocardiography allow improved evaluation of tricuspid valvar function. As a result, the heterogeneous category of congenital tricuspid valvar dysplasia may be better understood from a functional point of view. We are reporting a distinct entity in which tricuspid valvar regurgitation results from failure of coaptation due to short tendinous cords tethering the septal leaflet.

Patients and Results

Three children with significant primary tricuspid regurgitation were evaluated, treated, and followed. On echocardiographic evaluation, a central regurgitant jet of moderate or severe degree was directed toward the atrial septum through poorly coapting tricuspid valvar leaflets, which did not approximate due to tethering of the septal leaflet by abnormally short cords. In one patient, the tricuspid valve was otherwise normal; in the other two the leaflets and cords were also thickened. Two patients underwent surgery at 9 and 11 years of age. The cords tethering the septal leaflet were augmented by interposing appropriate lengths of expanded polytetrafluoroethylene suture and performing commissural annuloplasty. Both patients are asymptomatic 33 and 42 months postoperatively, with mild residual tricuspid regurgitation that has not changed since surgery. The other patient, an 8 month-old infant, has not yet undergone surgery.

Conclusions

Asymmetric tendinous cords of the tricuspid valve causing tethering of the septal leaflet is a distinct cause of tricuspid regurgitation that can be recognized with echocardiography. Although rare, the importance of recognizing this lesion lies in its being readily amenable to surgical repair.
Echocardiographic diagnosis of totally anomalous pulmonary venous connection to the azygos vein
Authors: Musolino Anna Maria , Santoro Giuseppe , Marino Bruno , Formigari Roberto , Guccione Paolo , Pasquini Luciano
Published: August 2008

Totally anomalous pulmonary venous connection to the azygos vein is a rare congenital heart malformation in which all the pulmonary venous blood returns anomalously to the azygos vein. Among 111 consecutive patients with totally anomalous pulmonary venous connection undergoing surgical correction at our institution between June 1982 and September 1997, this malformation was present in seven cases. By echocardiography, using a subxyphoid short-axis view at the atrial level and a modified suprasternal sagittal view, the malformation was diagnosed when the pulmonary venous confluence was traced posteriorly and superiorly relative to the right pulmonary artery and right bronchus, finally reaching reach the superior caval vein. Totally anomalous pulmonary venous connection to the azygos vein was misdiagnosed in the first two patients, both by echocardiography and angiocardiography. In the subsequent five patients, a precise diagnosis was obtained by echocardiography. Echocardiography, therefore, can be considered an accurate diagnostic tool permitting recognition of totally anomalous pulmonary venous connection to the azygos vein, and permitting corrective surgery without recourse to catheterization and angiography.
Isolated connection of the right superior caval vein to the left atrium: non-invasive neonatal diagnosis
Authors: Kakadekar Ashok P. , McKay Roxane , Tyrrell Michael J.
Published: August 2008

Isolated anomalous drainage of the right superior caval vein to the left atrium is a very rare cause of cyanosis in the newborn. Herein, the cross-sectional echocardiography and colour Doppler findings of this malformation are described.
Double-switch operation for congenitally corrected transposition and Ebstein's malformation
Authors: Nikoloudakis Nikolaos , Lindinger Angelika , Schäfers Hans-Joachim
Published: August 2008

An infant is described with congenitally corrected transposition and Ebstein's malformation. Banding of the pulmonary trunk had been previously performed because of a muscular ventricular septal defect. The patient underwent the double-switch procedure with the intention of unloading the morphologically right ventricle and the malformed tricuspid valve. This resulted in prompt postoperative functional and haemodynamic improvement.
An unusual type of combined aortic coarctation due to fibromuscular dysplasia
Authors: Suarez William A. , Kurczynski Thaddeus W. , Bove Edward L.
Published: August 2008

A 3-year-old patient is described with an unusual form of co-arctation due to hypoplasia of the transverse arch and fibromuscular dysplasia involving a long segment of the thoracic aorta. Surgical repair required resection of the aorta from the distal transverse arch to the mid-descending thoracic aorta, and replacement with a 16-mm Dacron interposition graft. This case demonstrates the importance of preoperative evaluation of the entire aorta in the presence of co-arctation due to fibromuscular dysplasia.
Lobar emphysema due to anomalous aortic origin of the left pulmonary artery
Authors: Absher Dale R. , Kriss Vesna Martich , Cottrill Carol M.
Published: August 2008

The unusual case of an infant with aortic origin of the left pulmonary artery is presented. The patient developed a rare complication of lobar emphysema due to bronchial compression from the enlarged right pulmonary artery. Operative anastomosis of the left pulmonary artery to the pulmonary trunk was successful, with subsequent resolution of the lobar emphysema.
Hypoplastic left heart syndrome with right aortic arch, bilateral arterial ducts and origin of the left subclavian artery from the left pulmonary artery
Authors: Patel Chandrakant R. , Specter Michael L. , Zahka Kenneth G.
Published: August 2008

The rare association, in a left-sided heart with hypoplastic left heart syndrome, of right aortic arch, bilateral patent arterial ducts and origin of the left subclavian artery from the left pulmonary artery are described. Cardiac catheterization was performed because of the abnormal anatomy of the arch noted at echocardiographic examination. This abnormality is of surgical importance when planning the Norwood operation.
Ascites and weight loss in a child: due to congenital division of the right atrium
Authors: Alkhulaifi Abdul M. , Serraf Alain , Planché Claude
Published: August 2008

Congenital division of an atrial chamber is a very rare congenital malformation that more commonly affects the left atrium but which may, in rare circumstances, involve the right atrium. Such a divided right atrium may present with symptoms consistent with increased portal venous pressure. Reported is a case with unusual clinical presentation. The patient underwent resection of the dividing shelf with good postoperative results.
A strange complex of diffuse congenital cardiovascular disease and cardiomyopathy, with localised myocardial calcification
Authors: Gerlis L. M. , Somerville J.
Published: August 2008

This unique case is of a man, followed clinically since infancy, who had a ventricular septal defect which closed spontaneously, a small arterial duct, and a minor degree of aortic coarctation, all without obvious symptoms. He later developed progressive cardiac failure which was attributed to some obscure form of congenital cardiomyopathy. He died at the age of 45 years. Necropsy showed a grossly abnormal arrangement of ventricular myocardial fascicles and bands, with absence of the papillary muscles causing tricuspid and mitral regurgitation. The various malformations are considered to be a gross example of a diffuse congenital cardiovascular disease complex, to the best of our knowledge previously undescribed.
Morphology of the elastic pulmonary arteries in pulmonary hypertension: a quantitative study
Authors: McLeod Karen A. , Gerlis Leon M. , Williams Gordon J.
Published: August 2008

Recent studies of intravascular ultrasound of the pulmonary arteries suggest that the technique can detect intimal and medial thickening in patients with pulmonary hypertension, potentially providing a method of assessing severity of pulmonary vascular disease in life. A major drawback of the technique is that only the elastic pulmonary arteries are accessible to current ultrasound catheters. The aim of this study was to determine whether morphological changes in vessels accessible to intravascular imaging reflect severity of pulmonary vascular disease and are of a sufficient degree to be detectable by current ultrasound catheters. Morphometric studies of the elastic pulmonary arteries were performed in specimens of lungs from 24 patients who had died with pulmonary hypertension (aged 3 weeks–9 years) and compared with measurements from infants who had died from sudden infant death syndrome. Morphological changes evident in the elastic pulmonary arteries in pulmonary hypertension included luminal dilation and medial thickening but these changes were too variable to be predictive of the severity of peripheral pulmonary vascular disease. Intimal thickening and atherosclerosis were present only in those with advanced pulmonary hypertensive disease. The changes of medial thickening and luminal dilation, nonetheless, are too variable to be predictive of the severity of peripheral pulmonary vascular disease. Thus, imaging by intravascular ultrasound may help confirm advanced pulmonary vascular disease, but is unlikely to differentiate less severe pulmonary vascular disease when the patient may still potentially be operable.
Transcatheter embolization of congenital coronary arterial fistulas in adults
Authors: Kambara Antônio M. , Pedra Carlos A. C. , Esteves C,sar A. , Cano Manoel N. , Braga Sérgio L. N. , Souza Amanda G. M. R. , Souza J. Eduardo M. R. , Fontes Valmir F.
Published: August 2008

In this report, we describe our experience with transcatheter occlusion of congenital coronary arterial fistulas in adults. From November 1992 to November 1996, 5 symptomatic patients, aged from 47 to 70 years, underwent transcatheter occlusion of fistulas using a retrograde arterial approach. All had chest pain or dyspnea on exertion. Detachable balloons were used in 4 patients, and Gianturco coils in 1. Detachable balloons were implanted through a Debrun system, while the coils were implanted through a 5 French right coronary Judkins catheter. Both were passed through an 8 French guiding catheter (Amplatz II). Each patient had a single fistula. The fistulas originated from the right coronary artery in 3 patients, and from the circumflex artery in 2. They drained into the pulmonary trunk in 3 patients, into the right atrium in 1, andinto a bronchial artery in the other. All fistulas were occluded completely in the catheterization laboratory, and the procedures were uncomplicated. At follow up, 3 patients underwent coronary angiography, and there was no evidence of recanalization. Transcatheter embolization in adults of single congenital coronary fistulas with detachable balloons and coils is safe and effective and can be regarded as an acceptable alternative to surgery.
Effect of radiofrequency catheter ablation of accessory pathways on autonomic tone in children
Authors: Sehra Ruchir , Hubbard Joyce E. , Straka Susan P. , Fineberg Naomi S. , Zipes Douglas P. , Engelstein Erica D.
Published: August 2008

Background

Radiofrequency catheter ablation is standard treatment for children with re-entrant supraventricular tachycardias. Autonomic changes have been noted after such ablation for atrioventricular nodal re-entry tachycardia, but not as well documented with atrioventricular re-entry over an accessory pathway.

Methods and results

In 10 normal paediatric volunteers and 12 children referred for electrophysiologic testing and radiofrequency ablation of supraventricular tachycardia, non-invasive autonomic function tests and tilt-table testing were performed, and the variability in 24-h heart rate was analysed. Patients with supraventricular tachycardia underwent these tests both 24–72 h before and 24 h after ablation. Patients with tachycardia underwent additional autonomic testing to assess the sensitivity of baroreceptors and the intrinsic heart rate with autonomic blockade immediately before and after ablation. One non-invasive autonomic function test, namely handgrip, demonstrated significant differences (p < 0.05) in diastolic blood pressure before and after ablation, though these values did not differ from controls. Significant decreases were noted in two indexes of the variability of heart rate before and after ablation (p < 0.05). Certain tilt test variables also demonstrated significant differences between controls and those with tachycardia subsequent to ablation. Intracardiac testing demonstrated changes (p < 0.05) in sinus cycle lengths, effective refractory periods and/or blood pressures at baseline and during testing of the sensitivity of baroreceptors before and after ablation. These changes were consistent with increased sympathetic or decreased parasympathetic tone. With autonomic blockade, these differences were abolished.

Conclusions

Catheter ablation of accessory pathways in children was associated with changes consistent with increased sympathetic or decreased parasympathetic tone. These autonomic changes persisted 24 h after the ablation procedure.
Right ventricular diastolic function after repair of tetralogy of Fallot: its relationship to the insertion of a ‘transannular’ patch
Authors: Eročlu Ayşe Güler , Sarioşlu Ayse , Sariočlu Tayyar
Published: August 2008

Examined was the effect of surgical technique, particularly the insertion of a transannular patch, on right ventricular diastolic function, and the relationship of forward flow in the pulmonary arteries during late diastole to right ventricular diastolic function in patients with tetralogy of Fallot. Transtricuspid, superior caval venous and pulmonary arterial Doppler spectrals were obtained and compared between 44 patients who had been repaired with a transannular patch; 14 patients who had been repaired with muscular resection and/or pulmonary valvotomy; six who had been repaired with an infundubular patch; and 32 normal children. The velocities of forward flow during late diastole in the pulmonary arteries of normal children ranged from 19.8 to 29.4 cm s−1(mean 24.9 ± 2.8 cm s−1) throughout the respiratory cycle. Restrictive right ventricular physiology, defined on the basis of increased forward flow in the pulmonary arteries during late diastole (> 30 cm s−1) was present in 25 (57°) of 44 patients with tetralogy of Fallot repaired using a transannular patch. Right ventricular volume was 50.1 ± 23.7 cm3in patients with a restrictive right ventricle and 64.9 ± 21.4 cm3in patients in whom the ventricle was non-restrictive (p< 0.03). QRS duration was 140 ± 18 and 156 ± 24 ms in patients with restrictive and non-restrictive right ventricular physiology respectively (p< 0.003). Restrictive physiology was not encountered in patients with tetralogy in whom the pulmonary valve had been preserved. It is concluded that right ventricular restriction is present in many patients with tetralogy of Fallot at mid-term follow-up subsequent to repair using a ‘transannular’ patch. Restriction is associated with smaller right ventricular size and less prolongation of the QRS complex.
Difficulties generated by the small, persistently patent, arterial duct
Authors: Raaijmaakers Brigitte , Nijveld Aagje , van Oort Anton , Tanke Ronald , Daniëls Otto
Published: August 2008

Over recent years, echo-Doppler cardiography has shown that a small, sometimes silent, arterial duct exists in more patients than previously recognized. To know the incidence of an arterial duct subsequent to therapy, we studied retrospectively our patients undergoing open-heart surgery and surgical or catheter closure. Three groups of patients were studied: those with patency of the duct subsequent to open heart surgery without any sign of patency before or during surgery, those with persistent duct after surgical ligation and those with persistent patency after attempted catheter occlusion with the Rashkind device. In the first group (of 431 children) four (0.9%) had persistence of this duct, of which three were silent. In the second group, patency persisted in four of 100 patients (4%), three being silent. In the last group there were five persisting shunts, three producing no murmur, in 30 patients (17%). We compared our results with those reported in the literature and conclude that echo-Doppler cardiography is needed to detect persistent shunting across a duct after therapy, since most of the residual ducts in this study were silent. This means that clinical findings alone cannot be relied upon, and careful echo-Doppler cardiography is essential. Also, the process of closure of a persistent duct by surgical ligation or transcatheter intervention is no guarantee of success. The risk of infective endocarditis is important in such persistent ducts and, at present, it is unknown either for a small, silent duct or in a persistent duct that remains open after attempted transcatheter closure, but now is in association with a foreign body.
Seasonality in live births with congenital heart disease in Malta
Authors: Grech Victor
Published: August 2008

This study was carried out to analyse seasonal variations in live births with congenital heart disease in the overall population of Malta. Included were all patients diagnosed as having congenital heart disease by echocardiography, cardiac catheterization, surgery or post mortem, by 1 year of age, and who were born between in Malta between 1990 and 1994. Results were analysed by χ2 and by Edwards' cyclic method. There were 231 cases of live born congenital heart disease, which were divided into 114 cases not requiring intervention and 117 cases requiring intervention. Although the overall prevalence of congenital heart disease did not demonstrate any seasonal variation, the lesions requiring intervention showed a significant peak in September by Edwards' method (p = 0.03), which was enhanced by seasonal analysis (p = 0.003). These results were confirmed by conventional χ2 and χ2 for trend. The lesions not requiring intervention, which were comprised almost entirely (96%) of mild pulmonary stenosis and small ventricular septal defects, failed to show any seasonal trend. The literature regarding such seasonal trends in these malformations and statistical analysis of seasonality of congenital heart disease are reviewed. An environmental factor such as a maternal viral infection or treatment of such infections during the first trimester of pregnancy from November to January may precipitate congenital heart disease in predisposed Maltese foetuses.
Relationship of the dimension of cardiac structures to body size: an echocardiographic study in normal infants and children
Authors: Daubeney P. E. F. , Blackstone E. H. , Weintraub R. G. , Slavik Z. , Scanlon J. , Webber S. A.
Published: August 2008

Normalization of the dimensions of cardiac structures to the size of the body, using so-called Z scores, is becoming increasingly common in the management of infants and children with congenital heart disease. Current published nomograms for the ascertainment of Z scores for cardiac structures in childhood are based largely on normal data obtained in formalin-fixed hearts. Since decisions concerning management are frequently based on the findings of cross-sectional echocardiograms, the dimensions of 15 cardiac structures were measured using cross-sectional echocardiography in 125 normal infants and children. Regression equations were derived relating cardiac dimensions to the size of the body. The expression of size with the highest correlation to cardiac dimensions was body surface area. Nomograms were then developed from which the Z score of a cardiac structure could be estimated from a knowledge of the body surface area and the echocardiographically derived measurement.
Double-outlet right ventricle with intact ventricular septum in a foetus with trisomy-18
Authors: Patel Chandrakant R. , Muise Kevin L. , Redline Raymond W
Published: August 2008

A rare case of double-outlet right ventricle with intact ventricular septum diagnosed by foetal echocardiography at 21 weeks of gestation is described. Amniocentesis revealed trisomy–18. The cardiac diagnosis was confirmed at autopsy.
Divided left atrium associated with supravalvar mitral ring
Authors: Álvares Silvia , Melo António Sá , Antunes Manuel
Published: August 2008

Reported is a case with a rare association of divided left atrium, supramitral stenosing ring of the left atrium, connection of the left superior caval vein to the roof of the left atrium, unroofed coronary sinus with an interatrial communication at the mouth of the unroofed sinus and ventricular septal defect. The need for a complete echocardiographic examination in the presence of pulmonary venous obstruction is emphasized. Surgery was successful in spite of significant preoperative pulmonary hypertension.
Divided right atrium. Diagnosis by echocardiography, and considerations on the functional role of the Eustachian valve
Authors: Corno Antonio F. , Bron Cédric , von Segesser Ludwig K.
Published: August 2008

A child presented at birth with severe cyanosis. Echocardiography showed hypoplasia of the right heart with a right-to-left shunt at atrial level. A conservative approach was adopted initially, and the situation improved over a few months, with reversal of the atrial shunt. Surgery was successfully performed at 4 years of age after further echocardiography revealed a congenitally large Eustachian valve and an atrial septal defect.
Sequential segmental approach to fetal congenital heart disease
Authors: Yoo Shi-Joon , Lee Young Ho , Cho Kyoung Sik , Kim Dae-Young
Published: August 2008

The sequential segmental approach is now universally used in the diagnosis of congenital heart disease. Its utilization during fetal sonography has not been well described. In this review, we show how the fetal heart can be approached in a sequential segmental manner by using six basic sonographic views. The transverse view of the fetal upper abdomen is obtained to determine the arrangement of the abdominal organs, which, in most cases, provides the important clues to the determination of the atrial arrangement. The four-chamber view is obtained to evaluate the atrioventricular junctions. The views of the left and right ventricular outflow tracts are obtained to evaluate the ventriculoarterial junctions. The three-vessel view and the aortic arch view are obtained for the evaluation of the arrangement and size of the great arteries, which provides the additional clues to the diagnosis of the abnormalities involving the ventriculoarterial junctions and the great arteries. The standard protocol to acquire these six basic views is also introduced.