bluuebunny/crossref_metadata_2025 huggingface dataset:
Interruption of the aortic arch associated with deletion of chromosome 22q11 is associated with a subarterial and doubly committed ventricular septal defect in Japanese patients
Authors: Momma Kazuo , Ando Masahiko , Matsuoka Rumiko , Joo Kunitaka
Published: August 2008

Objectives

The purpose of this study was to clarify the clinical characteristics of interruption of the aortic arch associated with chromosome 22q11 deletion.

Background

About half of patients with interruption of the aortic arch between the left common carotid and the left subclavian artery have deletion of chromosome 22q11.

Methods

In total, 20 patients with interruption of the aortic arch were studied with fluorescence in situ hybridization using peripheral lymphocytes and a DiGeorge syndrome chromosomal probe (Oncor N25). Cardiovascular anomalies in these patients were diagnosed by cross-sectional echocardiography and angiocardiography, and were confirmed at intracardiac repair.

Results

Of 13 patients with interruption between the left common carotid artery and the left subclavian artery, seven had the deletion. All 7 also showed thymic hypoplasia and hypocalcemia, together with a nasal voice and peculiar facies. Six of the seven patients had complete deficiency of the muscular outlet septum, with the defect extending to the perimembranous area. Such complete absence of the muscular outlet septum was not present in any of the patients without the deletion.

Conclusions

Interruption of the aortic arch between the left common carotid and the left subclavian artery, absence of the thymus, and complete absence of the muscular outlet septum, were characteristic in Japanese patients with interruption of the aortic arch associated with deletion of chromosome 22q11.
Transcatheter closure as standard treatment for most interatrial defects: experience in 200 patients treated with the Amplatzer ™ Septal Occluder
Authors: Berger Felix , Ewert Peter , Boöjrnstad Per G. , Dähnert Ingo , Krings Gregor , Brilla-Austenat Ira , Vogel Michael , Lange Peter E.
Published: August 2008

To judge whether an Amplatzer™ Septal Occluder can be used as standard therapy instead of surgery for closure of atrial septal defects we report our experiences in 200 patients. Of these patients, 127 had an atrial septal defect with haemodynamically significant left-to-right shunt, 68 patients a persistent oval foramen after presumed paradoxical embolism, and 5 had a fenestration after Fontan-repair. Mean age was 29.8 years (0.8 to 77.7 years). Body weight ranged from 6.9 to 120.0 kg (mean 51.5 kg). After diagnostic cardiac catheterization, and balloon-sizing of the defect, we implanted Amplatzer™ Septal Occluders with stents of 4 to 28 mm diameter. Follow-up studies were obtained after 48hours, and one, six, and twelve months. Transcatheter closure of the atrial septal defect proved successful in all without any relevant residual shunts. In particular, complete closure was achieved in all patients after presumed paradoxical embolism. The mean period of follow-up is 9–5 months, with a range from 0.4 to 23.5 months, giving a total of 1898 patient months. The occlusion rate after three month was 98.1°. A trivial haemodynamically insignificant residual shunt remained in 1.9° of the patients. Fluoroscopy times ranged from 0 to 43.5 minutes, with a median of 8.7 minutes. The excellent results in the short and medium term in children and adults have resulted in using this device routinely at the present time for closure of central atrial septal defects up to a diameter of 28 mm. Final judgement, however, is only possible after long-term follow-up.
Reduced frequency of occlusion of aorto-pulmonary shunts in infants receiving Aspirin
Authors: Motz Reinald , Wessel Armin , Ruschewski Wofgang , Bürsch Jochen
Published: August 2008

Objective

Infants with severely reduced pulmonary perfusion due to complex congenital cardiac malformations are in need of an improved flow of blood to the lungs. One option for treatment is to construct a systemic-to-pulmonary arterial shunt. Although such shunts have been used since 1945, their spontaneous occlusion remains a major problem in the long-term.

Design

We studied all infants in whom a systemic-to-pulmonary arterial shunt had been constructed using a Gore-Tex tube graft between December 1989 and March 1996.

Patients

Of 46 infants undergoing construction of a shunt, 7 (15°) died within 30 days of surgery. The shunts had to be taken down in 2 infants. Thus, 37 infants were included in the study. All but three infants received Aspirin. Aspirin was discontinued on the personal decision of individual physicians. Of 22 infants, 3 never received Aspirin, and in 19 it was stopped well before undertaking subsequent surgery. Aspirin was administered continuously to 15 infants until further surgery.

Results

Those in whom Aspirin was discontinued, or not given, and those receiving Aspirin until further surgery, were comparable concerning their age, time of follow-up, severity of the cardiac lesions, and size and type of shunt. Partial or complete occlusion of the shunt occurred in 2 of 15 (13°) infants taking Aspirin, but was seen in 12 of 22 (54°) infants in whom Aspirin was discontinued. Of these, 3 died due to acute occlusion of the shunt.

Conclusions

Aspirin reduced effectively the rate of occlusion of systemic-to-pulmonary arterial shunts, and should be continued as long as the shunt is in place.
Unguarded mitral orifice, mirror-imaged atrial arrangement, and discordant atrioventricular connections
Authors: Yasukochi Satoshi , Satomi Gengi , Park Insam , Ando Masahiko , Momma Kazuo
Published: August 2008

We report two autopsy proven cases of unguarded mitral orifice associated with mirror-imaged atrial arrangement, discordant atrioventricular connections, double outlet right ventricle, pulmonary valvar stenosis or atresia, and atrialisation of the morphologically left ventricle. The morphologically left atrioventricular junction was devoid of valvar leaflets, and there was no tension apparatus within the ventricle. To the best of our knowledge, this is the first description of this rare cardiac malformation
Comparison of acute effects on right ventricular haemodynamics of surgical versus interventional closure of atrial septal defects
Authors: Berger F. , Jin Z. , Ishihashi K. , Vogel M. , Ewert P. , Alexi-Meshkishvili V. , Weng Y. , Lange P. E.
Published: August 2008

Objectives

We compared the acute effects on right ventricular haemodynamics of surgical versus transcatheter closure of medium-sized atrial septal defects.

Methods

We studied 47 consecutive patients with a defect in the oval fossa and a ratio of pulmonary to systemic flows between 1.5: 1 and 2: 1. They were divided into two groups according to whether the defects were closed by surgery, performed in 23 patients, or by interventional catheterization, achieved in 24 patients. By means of transthoracic cross-sectional echocardiography, we measured right ventricular end-diastolic and endsystolic volumes and calculated ejection fractions. These calculations were performed before, and between 1 and 7 days after closure of the defect.

Results

Before closure of the defect, all patients had an enlarged right ventricle with normal function. After closure by either method, there was no difference in the rate of normalization of end-diastolic volume, but endsystolic volume remained enlarged. Thus, the calculated ejection fraction was lower than before closure.

Conclusions

There was no difference in right ventricular volumes or function early after closure of atrial septal defects, irrespective of whether this was achieved surgically or via transcatheter closure.
The aortic valve with two leaflets — a study in 2,000 autopsies
Authors: Pauperio Helder M. , Azevedo Arthur C. , Ferreira Claudia S.
Published: August 2008

Our study is based on the examination of 2,000 aortic valves obtained from fresh cadavers (1,499 males, 501 females) at the Institute of Forensic Medicine in Rio de Janeiro. We discovered 13 valves having two leaflets, giving a prevalence of 0.65%, much lower than generally reported in the literature. All 13 valves were from males, 10 from whites, three from mulattos, and none from blacks. Special attention was given to the raphe and the leaflets, the calcification of which can lead to stenosis. The only valves with normal texture and flexibility were two obtained from children less than one year old. All the other valves were thickened, and five of them had some degree of calcification. Six valves were judged to be functioning normally, while 7 valves were abnormal, 5 being stenotic and two showing evidence of insufficiency. The insufficiency in one was due to endocarditis, but in the other was due to redundancy of the leaflets. The only other cardiac anomaly discovered in these 13 cases was one patient with aortic coarctation. Bifoliate aortic valve, therefore, is probably the most common cardiac anomaly, although its prevalence as discovered in Brazil is lower than that reported in the literature. It affects mainly white males. After the fourth decade of life, most valves present some thickening, with stenosis being the most common complication. As is well recognised, infective endocarditis and aortic insufficiency are the other frequent complications. It is in general, nonetheless, an isolated anomaly.
The prevalence of bifoliate pulmonary valves
Authors: Gerlis L. M.
Published: August 2008

A BIFOLIATE CONDITION OF THE PULMONARY: valve may occur as an isolated phenomenon in otherwise normal hearts or in association with other anomalies in congenitally malformed hearts. The former condition is very rare, whereas the latter condition is quite common.
Spontaneous closure and thrombosis of a ductal aneurysm in a neonate
Authors: Maisel Peccina , Brenner Joel
Published: August 2008

The natural history of a ductal aneurysm detected prenatally is presented. Neurologic concerns in the neonate and absence of cardio-respiratory compromise permitted serial echo demonstration of constriction, thickening and subsequent closure with formation of thrombus in the aneurysm.
Cyanosis due to diastolic right-to-left shunting across a ventricular septal defect in a patient with repaired tetralogy of Fallot and pulmonary atresia
Authors: Thomson J. D. R. , Forster J. , Gibbs J. L.
Published: August 2008

Cyanosis as a result of right-to-left shunting across a ventricular septal defect is commonly encountered in patients with congenital heart disease when systolic pressure in the right ventricle exceeds that in the left ventricle. Reported is the case of a child who remained cyanosed after surgical correction of pulmonary atresia despite right ventricular systolic pressure being lower than left ventricular pressure. Colour-flow Doppler showed a residual ventricular septal defect, with right-to-left shunting in diastole alone.
Obstruction of the right ventricular outflow tract caused by a tuberculoma in a patient with ventricular septal defect and aneurysm of the membranous septum
Authors: Santos Cleusa Lapa , Moraes Fernando , Moraes Carlos R.
Published: August 2008

Reported here is an obstruction of the right ventricular outflow tract caused by a tuberculoma in a 15-year-old boy who presented with a ventricular septal defect. The obstruction was discovered at surgery and the tuberculous aetiology was only demonstrated histologically. To the authors' knowledge, this is the first report of a tuberculoma of the heart associated with congenital heart disease.
Pulmonary atresia with intact ventricular septum, right-sided aortic arch, and an aorto-pulmonary collateral artery
Authors: Patel Chandrakant R. , Spector Michael L. , Zahka Kenneth G.
Published: August 2008

Described is a rare association in a patient with the heart in the left chest, namely pulmonary atresia with intact ventricular septum, fistulous coronary arterial connections, a right-sided aortic arch and an aorto-pulmonary collateral artery feeding one lung. The pulmonary arteries were non-confluent, with the right lung supplied by the right arterial duct originating from the under surface of the right-sided aortic arch, and the left lung supplied through the aorto-pulmonary collateral artery arising from the descending aorta. The surgical management is different in the setting of non-confluent pulmonary arteries.
Interruption of the aortic arch at the isthmus with DiGeorge syndrome and 22q11.2 deletion
Authors: Takahashi Kazuhiro , Kuwahara Takashi , Nagatsu Masayoshi
Published: August 2008

A 6-day-old male with interruption of the aortic arch at the isthmus (type A) had the typical phenotype of DiGeorge syndrome. There was also a doubly committed juxta-arterial ventricular septal defect and an unobstructed left ventricular outflow tract. Hypoplasia of the thymus was confirmed during a modified Blalock-Park operation. He had persistent hypocalcemia, and was susceptible to infection. He was subsequently revealed by the use of fluorescence in situ hybridization analysis to have 22q11.2 deletion. Interruption of the aortic arch at the isthmus is presumed to reflect abnormal fetal hemodynamics, and is considered a distinct pathogenetic entity from interruption between the left common carotid and subclavian arteries, the latter being the variant more frequently associated with DiGeorge syndrome. In our case, the 22q11.2 deletion likely played a major role in the etiology of the interrupted aortic arch.
Rapid reversal of dilated cardiomyopathy following removal of neuroblastoma
Authors: Rosti Luca , Festa Pierluigi , Corbetta Carlo
Published: August 2008

Reported is a child with dilated cardiomyopathy, in whom medical therapy resulted in a mild improvement of cardiac function. Metabolic studies suggested the presence of a catecholamine-secreting tumour; and an adrenal neuroblastoma was identified and surgically removed. Following surgery, there was progressive and complete normalization of cardiac function. Although very rare, neurogenic tumours may be involved in the development of a dilated cardiomyopathy in the infant and child.
Cardiac anomalies associated with congenital absence of the portal vein
Authors: Massin Martial , Verloes Alain , Jamblin Paul
Published: August 2008

The authors discovered congenital absence of the portal vein, with visceral venous return to the right atrium, in a 5-year-old girl with aortic valvar stenosis. Interestingly, of the 19 patients, it was discovered that 11 reported with portal venous agenesis also had cardiac defects. We have, therefore, investigated the hypothesis that the congenital absence of the portal vein and the associated cardiac malformations may result from a similar embryologic insult, and that cardiac development may be affected by the systemic diversion of portal venous flow.
The syndrome of pericarditis, arthritis, and camptodactyly: an under-recognized cause of pericardial constriction in children?
Authors: Buendía Alfonso , Attié Fause , Martínez-Lavin Manuel
Published: August 2008

The syndrome encompassing the combination of pericarditis, arthritis, and camptodactyly is a rarely described cause of pericardial constriction in children. It is likely that this association is being under-recognized. We report a new case in which the skeletal abnormalities were subtle. The syndrome should be included in the differential diagnosis of any child with persistent non-inflammatory pericardial effusion. A careful search at the bedside for the associated skeletal abnormalities should lead to the correct diagnosis. Pericardiectomy is the treatment of choice.
Magnetic resonance imaging comes of age
Authors: Razavi Reza , Baker Edward
Published: August 2008

MAGNETIC RESONANCE IMAGING OF THE heart started in the 1970's, over the subsequent 25 years, enormous advances have been made in both the hardware and software of magnetic resonance imaging machines so that we can now obtain fast, detailed and accurate images of the heart and great vessels. In chronological terms, therefore, we can say that cardiac magnetic resonance imaging has come of age. In this article we will highlight some of the clinical applications of different magnetic resonance imaging techniques, as well as some recent developments. We will demonstrate that, in clinical investigation of congenital heart disease, magnetic resonance imaging has truly come of age.
Noonan syndrome – then and now
Authors: Noonan Jacqueline
Published: August 2008

The report by Danetz, Donofrio and Embrey1 provides an interesting long term follow-up on the first patient diagnosed by me with what is now known as Noonan syndrome. In 1960, when this patient was seen, cardiac ultrasound was unavailable and left-sided cardiac catheterization uncommon. He had severe pulmonary stenosis, and did well following surgery. Unfortunately, his follow-up was not adequate and left-sided obstruction was not recognized until he became symptomatic some thirty years later. Early reports stressed the dysplastic pulmonary valve, with pulmonary stenosis as a common finding.2 By 1972, the report of Ehlers et al.3 brought attention to the left-sided involvement in the Turner phenotype.
Transthoracic 3-dimensional echocardiography in the assessment of subaortic stenosis due to a restrictive ventricular septal defect in double inlet left ventricle with discordant ventriculoarterial connections
Authors: Vogel Michael , Ho S. Yen , Anderson Robert H. , Redingtont Andrew N.
Published: August 2008

To evaluate the accuracy and clinical utility of three-dimensional echocardiography in the assessment of the size and shape of the ventricular septal defect in double inlet left ventricle.

Methods

We validated the technique in an autopsy study, and then performed a clinical investigation. Six autopsied hearts were immersed in a waterbath and examined with 3-dimensional echocardiography. We identified the cross-section within the dataset which optimally displayed the ventricular septal defect “en face”, and compared its smallest and largest diameters, as well as its area. The ventricular septal defect was then filled with a silicone sealant and a section prepared for direct measurement. In patients, we measured the diameters and area of the ventricular septal defect in endsystole nad computed the aortic valvar area in endsystole from the cross-section showing the aortic valve “en face”. Ten patients with double inlet left ventricle, aged between 2 and 15 years, were studied using rotational or parallel scanning. All patients had undergone banding of the pulmonary trunk at a mean age of 7 (3–36) days, usually at the time of repair of the coarctation. Two patients had undergone surgical enlargement of the ventricular septal defect prior to echocardiographic examination.

Results

The correlation between the areas of the ventricular septal defect in the specimens measured directly and by 3-dimensional echocardiography was r=0.98, with limits of agreement between −0.1– 0.08 cm2. In the patients, the area of the defect was measured as 3.9±2 cm2, whereas the aortic valvar area was 2.6±0.9 cm2. The ratio between the areas was 1.5 (0.5–2.3). Three patients with areas of the ventricular septal defect smaller than those of the aortic valve had resting Doppler gradients between double inlet left ventricle and the aorta of 16, 20 and 30 mm Hgs, respectively.

Conclusions

3-dimensional echocardiography provides accurate assessment of the area of the ventricular septal defect in double inlet left ventricle, and is helpful in identifying patients with subaortic stenosis caused by restrictive defects.
Normal values of signal-averaged electrocardiographic parameters and QT dispersion in infants and children
Authors: Vialle Emmanuelle , Albalkhi Rim , Zimmerman Marc , Friedli Béat
Published: August 2008

Ventricular late potentials, and dispersion of the QT interval, are markers for risk of ventricular arrhythmias. Normal values for these parameters are well established in adults, but may not apply for children. This study has investigated the age dependency of signal averaged electrocardiographic parameters and QT dispersion in 111 normal children aged from 5 days to 16 years. The results indicate that parameters change with age: duration of filtered QRS and low amplitude (< 40 μV) terminal signal increase with age, especially in the younger patients. Filtered QRS is 88.9 ± 7.87 ms in infants, and increases to 108.7 ± 8.51 in teenagers (p<0.001). Low amplitude terminal signals are 17.0 ± 3.44 ms in infants, and 24.5 ± 5.64 ms in teenagers (p< 0.001). Root mean square of the last 40 ms decreases with age, but remains stable after the age of 10 years (122.4 ± 33.30 μV in infants, 60.9 ± 31.27 in teenagers,p<0.001). QT dispersion, on the other hand, does not change significantly with age. The mean value for the whole group is 36 ± 13.7 ms. A weak but significant correlation exists between QT dispersion and filtered QRS. Thus, age must be taken into consideration when interpreting signal-averaged electrocardiograms, but not when measuring QT dispersion.
Echocardiographic assessment of interrupted aortic arch
Authors: Kaulitz Renate , Jonas Richard A. , van der Velde Mary E.
Published: August 2008

Background

In patients with interrupted aortic arch echocardiography provides detailed information about the anatomy of the aortic arch and the associated cardiac anomalies. Only a few reports have evaluated the reliability of this non-invasive diagnostic procedure by correlation with angiographic and surgical findings.

Methods

From 1988 through 1993, 45 infants with interrupted arch underwent surgical repair (mean age 13.02 days). Of the patients, 33 had interruption of the arch between the left common carotid and subclavian arteries; 25 patients had a ventricular septal defect, and the remaining 20 had coexisting complex congenital heart defects. Preoperative diagnosis was made exclusively by echocardiography in 25 of the patients. Accuracy of echocardiographic diagnosis was evaluated retrospectively by comparing preoperative studies with angiography and surgical reports. We then investigated whether the morphologic features of the interrupted arch might influence surgical procedure or outcome.

Results

Intracardiac anatomy was accurately diagnosed by echocardiography in all cases; in 2 patients angiography provided additional information concerning the morphology of the aortic arch. Operative notes described differences in morphology of the arch in 7 patients, but these did not influence the surgical procedure. Direct anastomosis of the interrupted segments was possible in 38 patients, and 36 patients underwent primary intracardiac repair. Echocardiographic measurements revealed that the diameter of the ascending aorta was related to the number of vessels originating from the proximal aortic arch. The distance between the interrupted segments was significantly different according to the site of interruption, but not between cases with an isolated ventricular septal defect versus those with complex heart disease. It did not influence the method of arch repair, nor was it related to recurrent or residual obstruction.

Conclusion

Preoperative echocardiography offers accurate and complete diagnosis in the critically ill neonate with interrupted aortic arch and associated intracardiac abnormalities.
A 5-year experience with surgical repair of atrial septal defect employing limited exposure
Authors: Khan Junaid H. , McElhinney Doff B. , Reddy V. Mohan , Hanley Frank L.
Published: August 2008

Background

There has been a trend in recent years towards less invasive therapy for many congenital cardiac malformations. For the past 5 years, we have employed a technique of limited surgical exposure when repairing atrial defects within the oval fossa.

Methods

Over the 5-year period from July 1992 to August 1997, 115 consecutive patients underwent surgical repair of an isolated atrial septal defect in the region of the oval fossa by a single surgeon. The patients had a limited midline skin incision starting at the line of the nipples and extending inferiorly across 2 to 3 intercostal spaces. A partial sternotomy was performed, sparing the manubrium. Standard instruments and cannulation techniques were used for cardiopulmonary bypass and fibrillatory arrest.

Results

There were no deaths and no major complications. The median time to extubation after leaving the operating room was 3 hours (30 minutes to 8 days). Mediastinal drains were removed the morning after surgery. The median stay in the intensive care unit was 7 hours (3 hours to 10 days), and patients were discharged from the hospital a median of 4 days postopera-tively (2 to 23 days).

Conclusions

This approach using limited exposure can be applied safely without any new instruments and without peripheral incisions or sites of vascular access, while providing a comfortable exposure for the surgeon and achieving a cosmetically superior result for the patient.
Volumetric analysis of the right ventricle in children with congenital heart defects: comparison of biplane angiography and transthoracic 3-dimensional echocardiography
Authors: Heusch A. , Rübo J. , Krogmann O. N. , Bourgeois M.
Published: August 2008

Background

Three-dimensional echocardiography is a non-invasive imaging technique. The fact that it permits volumetric analyses independently of geometrical assumptions makes it a putatively useful method for the precise measurement of the volumes of the irregularly shaped right ventricles in children. The aim of this study was to assess the feasibility of this method and its agreement with angiocardiography based estimates of right ventricular volume in children with congenital heart disease.

Methods

We studied 102 children with congenital heart disease. The angiocardiographic right ventricular volumetry was performed using a biplanar technique using Simpson's rule and corrected with Lange's correction factors. The echo data sets were registered trans-thoracically with a rotating transmitter. Volumes were calculated after manual planimetry by adding the volumes of the individual slices.

Results

Calculation of right ventricular volume echocardiographically was possible only in 34% of patients, mostly infants and toddlers. In comparison to angiocardiography, the measured volumes were 1.1 ±6.9 ml (19.5 ±34.1%) or 6.3±9.4ml (42.5±33.6%) smaller during systole or diastole, respectively. The limits of agreement were −12.5 and 13.6ml, or 12.45 and 25.15ml during systole or diastole, respectively. When plotted to a logarithmical scale, the correlation coefficients r2 were 0.70 for systolic and 0.79 for diastolic measurements.

Conclusion

Transthoracic 3-dimensional echocardiography with a rotating transmitter is feasible for volumetry only in small children. The volumes measured were significantly smaller than the ones calculated from the angiocardiographic images. The correlation between the two methods is moderate.
Long-term follow-up of stents implanted to relieve peripheral pulmonary arterial stenosis: hemodynamic findings and results of lung perfusion scanning
Authors: Spadoni Isabella , Giusti Sandra , Bertolaccini Pietro , Maneschi Alberto , Kraft Giuliano , Carminati Mario
Published: August 2008

In recent years, percutaneous placement of stents has been used as an alternative to surgery or balloon angioplasty for the treatment of adults with peripheral pulmonary arterial stenosis. This therapy has also been proposed for children, but questions still remain about its indications in this group of patients. We describe here the results of intravascular placement of stents in a group of 29 patients, with a mean age of 12±7 (range 3–31) years and weighing 35 ±19 (range 11–74) kg. All were affected by postsurgical or congenital isolated pulmonary arterial stenosis, and have now been followed for 38 ±19 (range 6–65) months. The early hemodynamic results have been excellent, with a significant reduction of the pulmonary arterial systolic pressure, the systolic pressure gradient, and the ratio of systolic pressures in the pulmonary and systemic circuits, and with a significant increase of the diameter of the stented vessels in all the patients. Of the 29 patients, 24 have been recatheterized 18 ± 10 months after the procedure, demonstrating the stability of the results, with a low incidence of late restenosis, this seen in only 1 patient (2°). Lung perfusion scanning, performed in 17 patients each year after the follow-up catheterization, has showed that the results are maintained at long-term follow-up (51 ±9 months).
Changes of management in a patient with double outlet left ventricle
Authors: Galal Omar , Hatle Liv , Halees Zohair Al
Published: August 2008

Double outlet left ventricle is an extremely rare anomaly. Until recently, the diagnosis was usually established by angiography or at postmortem. There are only a few reports describing the echocar-diographic findings in this lesion, and as far as we know, no report showing the anatomy as well as the velocity and pattern of flow by color Doppler echocardiography. The patient reported here underwent surgery at the age of four years, when an aortic homograft was placed between the right ventricle and the pulmonary trunk. This biventricular repair had to be changed into a Fontan-type procedure, 15 years later since the hypoplastic right ventricle did not grow adequately.
A unique case of ventricular isomerism?
Authors: Rinne Katy , Smith Audrey , Ho Siew Yen
Published: August 2008

Isomerism of the atriums has often been described with various complex cardiac malformations. As far as is known, a case of ‘ventricular isomerism’ has never been recorded. Described is a specimen where, on the basis of morphological criterions, there are two right ventricles.
Multiple left-sided cardiac lesions in one of Noonan's original patients
Authors: Danetz Jeffrey S. , Donofrio Mary T. , Embrey Richard P.
Published: August 2008

We describe a complex case of obstruction of the left ventricular outflow tract in one of Dr. Noonan's original patients. Intraoperative findings revealed pathology at the valvar, subvalvar and supravalvar positions. Patients with Noonan syndrome are traditionally described as having right-sided cardiac pathology. Review of the literature revealed left-sided lesions to occur in a substantial number of these patients. We therefore suggest the routine employment of cardiac ultrasonography in all patients with Noonan syndrome with attention directed toward left-sided pathology, as well as the frequent pulmonary valvar pathology.
Common arterial trunk associated with atrioventricular septal defect
Authors: Atik Edmar , Soares Andressa Mussi , Aiello Vera Demarchi
Published: August 2008

Described are the clinical, cross-sectional echocardiographic, electrocardiographic and angiographic findings, together with the results at autopsy, in a 5-month old infant with a common arterial trunk associated with an atrioventricular septal defect. As far as is known, this is only the 13th such case to be reported in the literature. A particularly unusual feature of this case was an intact atrial component of the atrioventricular septal defect.
Successful radiofrequency ablation in an infant with drug-resistant permanent junctional reciprocating tachycardia
Authors: Jaeggi Edgar , Lau Kai-Chiu , Cooper Stephen G.
Published: August 2008

Over the past decade, the technique of radiofrequency ablation has evolved substantially. Currently, most forms of cardiac arrhythmias seen in children can be treated with good long-term results and low risk of adverse outcome. Curative arrhythmia treatment with this technique, however, is still uncommon in neonates and infants. Reported here is our experience in the management of an 8-week-old with drug-resistant permanent junctional reciprocating tachycardia.
Double outlet left ventricle with subpulmonary ventricular septal defect and pulmonary hypertension
Authors: Kiliç Ayhan , Saraçlar Muhsin , Özkutlu Süheyla
Published: August 2008

A two-month old male infant with the rare occurrence of double outlet left ventricle, subpulmonary ventricular septal defect and pulmonary hypertension is presented. The infant was managed temporarily with banding of the pulmonary trunk, with a favorable result, and is scheduled for definitive intraventricular repair.
The genetics of hypoplastic left heart syndrome
Authors: Grossfeld Paul D.
Published: August 2008

Hypoplastic left heart syndrome is one of the most therapeutically challenging congenital cardiac defects. It accounts for as many as 1.5% of all congenital heart defects, but is responsible for up to one quarter of deaths in neonates with heart disease.1The management of hypoplastic left heart syndrome is controversial. Two surgical options exist:2,3the Norwood procedure, is a three stage repair in which the morphologically right ventricle is converted to function as the systemic ventricle. Alternatively, orthotopic transplantation can be performed. Although both surgical options have had improved outcomes, the prognosis for long-term survival is guarded, with a five year survival for either approach reported to be in the region of 50–60%. In this review, I explore the evidence for a genetic etiology for the “classic” hypoplastic left heart syndrome, defined as mitral and/or aortic atresia with hypoplasia of the left ventricular cavity and the other left-sided structures.
Surgical treatment of congenital mitral valvar insuffciency: “The Hôpital Broussais” experience
Authors: Chauvaud Sylvan M. , Milhaileanu Serban A. , Gaer Julian A. R. , Carpentier Alain C.
Published: August 2008

There are many congenital malformations of the mitral valve which produce valvar insufficiency. From a surgical point of view, systems based exclusively on anatomic analysis are not always entirely appropriate for assessment of these lesions. With this in mind, Carpentier proposed a functional approach for analysis based upon the motion of the valvar leaflets. From 1969 to 1994, 135 children under the age of 12 (mean age: 5.8 + 3.15 Y, 0.6–12Y) underwent surgery in our department, basing treatment on such analysis. Since motion of the leaflets during the operation is compromised by cardioplegia, and sometimes exposure can be however difficult, preoperative echocardiography was a mandatory part of the diagnostic cascade.

Normal motion of the leaflets was present in 41 patients, with deformation of the annulus in 14, a cleft in 21, and partial agenesis in 6. Prolapse of leaflets was present in 42 patients. Leaflet motion was restricted in 28 patients. These were divided in two groups, one with normal papillary muscles and commissural fusion or short cords. The other with abnormal papillary muscles producing a parachute arrangement in 6 and a hammock valve in 9. Associated lesions were present in 47% of the patients. Conservative surgical procedures following the precepts developed by Carpentier were used in 127 patients. Valvar replacement was necessary in 8 patients. Operative mortality was 4%. Mean follow up was 8.4 ± 5.3 years (1–23Y). Actuarial survival at 5 years was 90 ± 6% and, at this time, was stable. No thromboembolic events occurred after conservative surgery. The reoperation rate was 5% for those undergoing repair (6 patients). We conclude that the functional classification developed by Carpentier is a reliable and robust approach to these complex lesions. Conservative surgery is feasible in most of the cases presenting with congenital mitral valvar insufficiency. Results are stable and reliable. Surgery should be undertaken before the onset of left ventricular deterioration.
Surgical treatment of congenital mitral valvar stenosis: “The Hôpital Broussais” experience
Authors: Chauvaud Sylvain M. , Milhaileanu Serban A. , Gaer Julian A. R. , Carpentier Alain C.
Published: August 2008

Lesions producing congenital mitral valvar stenosis are complex and often multiple, From 1970 to 1994, 58 patients under 12 years of age (mean 5 ± 3.2 y) underwent surgical treatment for such malformations in our department. In order to provide a clear understanding of the pathology, we used a classification based on anatomy and function. Echocardiography was a major tool of the preoperative investigations. The lesions were classified as follows:

Normal papillary muscles: commissural fusion in 19, excess leaflet tissue in 2, valvar ring in 12, and annular hypolasia in 2.

Abnormal papillary muscles: paachute arrangement in 10 and hammock mitral valve in 13.

Associated malformations were found in 63%, the most common being multiple stenosis in the left heart. Conservative treatment was possible in 54 patients (95%) using the techniques developed by Carpentier. Hospital mortality was 22%, with 95% confident limits between 13 and 36% (13/58). Of the 13 patients with a hammock valve, 6 died in hospital. The overall mean period of follow up was 7.8 + 5 years. No thromboembolic events occured after conservative surgery. Late death occured in 3%. The actuarial survival at 5 years was 64 + 15%. Reoperation was needed for residual mitral valvar stenosis in three patients or insufficiency in one.

In spite of effective techniques to relieve mitral obstruction, 12% of our patients need early or delayed replacement of the valve. The hospital mortality remains high due to the complexity of the lesions, associated with the associated malformations. Echocardiography proved to be extremly helpful for the functional analysis. In order to improve operative results, simple and early valvar repair, even if considered palliative, seems to be appropriate.
Surgical treatment of double outlet right ventricle
Authors: Burakovsky V.I. , Podzolkov V.P. , Ivanitsky A.V. , Ragimov F.R.
Published: August 2008

Form 1980 until January 1991, 76 patients with double outlet ventricle, aged from 1 year 7 months to 23.5 years (mean age 7.5± 5.0 years) underwent surgey at the A.N. Bakoulev Inste for Cardivascular Surgery. Associated heart defects were found in 77.6% patints. In 57 patients (75%), the left ventricle opened into the subaortic infundibulum; into the subpulmonary infudibulum in four (the Tassisg-Bing anomaly) while, in 12 (15.8%), it opened under the outflow tracts of both great arteries. In two cases, (2.6%) there was a mno-committed ventricular septact. In one case (1.3%)a non-committed defect was diagnosed as coexisting with a non-commited defect was dignosed as coexisting with a Taussig-Bing anomaly. There were eight operative deaths (10.9%) among the patients undergoing radical surgery.Another theree patients with the Taussig-Bing anomaly and obstructive pulmomnary vascular disease underwent a successful palliative Mustard operation. All deaths were associated with acute heart failure and were seen only among patiens with subaortic ventricular septal defects. The important factors, which increased the risk of correction and contributed to death were inappropriate pre-operative diagnosis of complex associated defecrs and techmical mistakes during surgical intervention, the latter due to inadequate surgical experience. During the last five years, mortality decreased significatly (one death among 34 patients undergoing surgery from 1986 through January 1991)
Supravalvar pulmonary stenosis following the arterial switch operation for complete transposition: aetiological and surgical considerations
Authors: Testolin Luca , Stellin Giovanni , Bianco Roberto , Rubino Maurizio , Michielon Guido , Salviato Nicoletta , Milanesi Ornella , Moreolo Giorgio Svaluto , Casarotto Dino
Published: August 2008

From January 1988 through to July 1994, 54 consecutive infants underwent an arterial switch operation for simple or more complex forms of complete transposition (concordant atrioventricular and discordant ventriculo-arterial connections). They ranged in age from 2 to 180 days. The Lecompte maneuver was performed in all. In the first 19 patients the harvested sinuses of Valsalva were filled with two separate patches of autologous preserved pericardium, while, in the last 35 patients, a wide pantaloon patch of tanned pericardium was employed. Five babies died within 30 days after the procedure (operative mortality of 9.2%, 70% CL 4–17%). Four survivors were found to have developed a significant supravalvar pulmonary stenosis from 1 month to 21 months postoperatively. Echocardiographic data showed a transpulmonary peak systolic gradient from 60 mmHg to 101 mmHg, with a right-to-left ventricular systolic pressure ratio from 0.65 to 0.9. Reoperation was performed from 8 months to 39 months after the arterial switch procedure. Supravalvar pulmonary stenosis was located at the level of the pulmonary trunk, extending distally in two cases, due to the growth of fibrous scarring tissue with partial calcification. The pulmonary valvar leaflets were involved in two cases. Relief of the obstruction was obtained by insertion of a wide shield-tailored polytetrafluoroethylene patch after making an inverted Y-shaped longitudinal incision in the pulmonary trunk between the anterior sinuses of Valsalva. No mortality occurred at reoperation. Early and midterm echocardiographic measurements showed the effectiveness of this technique, with only trivial or mild residual transpulmonary gradients.
Formation of thrombus in the residual pulmonary trunk and regurgitation of the pulmonary valve after total cavopulmonary connection
Authors: Hofbeck Michael , Singer Helmut , Buheitel Gernot , Sunnegardh Jan , Emde Jürgen V.D.
Published: August 2008

The procedure of total cavopulmonary connexion includes trans-section and closure of the pulmonary trunk, the systemic venous blood flow being directed to the pulmonary vascular bed by an anastomosis of the superior caval vein with the right pulmonary artery. Following this procedure, there remains a small residual pulmonary trunk. The purpose of our study was to obtain information about the fate of this residual trunk and its contained pulmonary valve. Using transthoracic echocardiography, we examined 29 patients (mean age 7 years 4 months) after a total cavopulmonary connexion (mean interval 17 months). We were able to obtain adequate visualization of the pulmonary valve and the pulmonary trunk in 23 of the patients. Pulmonary regurgitation was demonstrated by colour Doppler echocardiography in 13 pts (57%). Formation of thrombus in the residual pulmonary trunk was detected in one patient (4%) 6 months after the operation. One cerebrovascular embolic event occurred in a patient who had pulmonary regurgitation without formation of thrombus in the residual trunk 3 months after the total cavopulmonary connexion. Our findings show that pulmonary regurgitation is frequently present in patients after total cavopulmonary connexion. Although formation of thrombus in the residual pulmonary trunk seems to be less common, we recommend surgical closure of the pulmonary valve during this procedure so as to exclude the residual pulmonary trunk as a possible source of systemic emboli.
Bidirectional cavopulmonary shunt with an additional source of pulmonary flow: an interim or final stage of palliation
Authors: Day Ronald W. , Baker Charles M. , Caton John R. , Hawkins John A. , McGough Edwin C.
Published: August 2008

A bidirectional cavopulmonary shunt is performed in patients with single ventricle physiology to improve or maintain systemic oxygenation while decreasing the workload of the heart. During a period of 10 years, bidirectional cavopulmonary shunts were performed in 50 patients at our institution. The procedure was performed with an additional source of pulmonary flow through the pulmonary valve or a systemic to pulmonary shunt in 27 patients and without an additional source of pulmonary flow in 23 patients. Preoperative and postoperative chest radiographs, pulmonary angiograms, oxygenation, hemodynamic measurements, morbidity, and mortality were reviewed to determine whether a bidirectional cavopulmonary shunt with an additional source of pulmonary flow is an acceptable interim or final stage of palliation. Systemic oxygenation was improved only in patients with an additional source of pulmonary flow. The outcome of the bidirectional cavopulmonary shunt was not adversely affected by the presence of additional pulmonary flow. Twenty-five patients subsequently underwent an atrial to pulmonary or total cavopulmonary anastomosis. Oxygenation was improved by more definitive palliation; however, late complications of stroke, protein losing enteropathy, and arrhythmias requiring pacemaker therapy were more prevalent. In conclusion, systemic oxygenation can be improved by performing the bidirectional cavopulmonary shunt with an additional source of pulmonary flow through the pulmonary valve or a systemic to pulmonary shunt without an adverse effect on hemodynamic measurements, pulmonary arterial growth, morbidity, or survival. The bidirectional avopulmonary shunt may be an acceptable final stage of palliation in some patients with a functionally single ventricle.
The use of a newly developed pediatric arterial perfusion cannula for neonates
Authors: Asou Toshihide , Yasui Hisataka , Kado Hideaki , Imoto Yutaka , Shiokawa Yuichi , Fukae Kouji , Tominaga Ryuji , Kawachi Yoshito
Published: August 2008

We report our experience using an originally designed thin walled metal-tipped arterial cannula for neonatal open heart surgery. Its external diameter is 2.1 mm and its internal diameter is 1.7 mm, it has a high internal – external diameter ratio. It was used on 36 patients, who were either neonates or small infants. In 32 of the patients, it was inserted into the ascending aorta for systemic perfusion. In the other 4, it was inserted into the brachiocephalic artery for selective cerebral perfusion during repair of the aortic arch. The relationship between the pressure drop and the bypass flow was obtained for the 7 most recently treated patients. The pressure drop at a bypass flow of 500 ml/min was 150 mmHg. Due to the high internal – external diameter ratio of this cannula, and its excellent flow characteristics, we consider it to be particularly useful in neonatal open heart surgery, while it also allows for successful selective cerebral perfusion during repair of the aortic arch in neonates.
Balancing pulmonary and systemic arterial flows in parallel circulations: the value of monitoring system venous oxygen saturations
Authors: Riordan Christopher J. , Randsbaek Flemming , Storey John H. , Montgomery William D. , Santamore William P. , Austin Erle H.
Published: August 2008

Accurate bedside assessment of the ratio of pulmonary to systemic flow (Qp/Qs ratio, referred to as “the flow ratio” or “the ratio”) plays an important role in the management of many congenital heart defects, especially the complexes unified by univentricular atrioventricular connections. Arterial oxygen saturation can be a misleading measure of the ratio, and may not reflect derangements until they are quite large. Theoretical analysis suggests that systemic venous oxygenation may be a better indicator of the ratio. To examine this, we created a widely patent atrial septal defect in neonatal piglets (weight =4–6.5 kg, n=6). Snares aruond the aorta and pulmonary trunk were adjusted to alter the flow ratio from 0.1 to 6.5. Venous oxygen saturations, measured in the mid-inferior caval vein, were at a maximum at a ratio about 1, and declined rapidly with increases or decreases in the ratio beyond a limited range. The venous oxygen saturation was found to vary much more than arterial oxygen saturation, with arterial oxygen saturation only falling when the ratio dropped below 0.5. Oxygen delivery (Oxygen Content x Cardiacoutput) was found to parallel closely systemic venous oxygen saturation, and was at a maximum at the same ratio that produced a maximum value of systemic venous oxygen saturation. The study suggests that systemic venous oxygen saturation provides a better estimate than does systemic arterial oxygen saturationof the flow ratio and oxygen delivery. Interventions that maximize systemic venous oxygen saturation should maximize oxygen delivery, and determination of systemic venous oxygen saturation should be a helpful addition in managing children with a number of congenital heart defects.