bluuebunny/crossref_metadata_2025 huggingface dataset:
A prospective, randomised trial evaluating transatrial and transventricular approaches to repair of tetralogy of Fallot
Authors: Kaushal Sunil K. , Sharma Rajesh , Iyer Krishna S. , Kothari Shyam Sunder , Venugopal Panangipalli
Published: August 2008

The traditional approach to repair of tetralogy of Fallot involves a right ventriculotomy for closure of ventricular septal defect. During the past two decades, reports of progressive right ventricular dilation and dysfunction, and late occurrence of ventricular arrhythmias, have led investigators to re-evaluate this approach and advocate instead the transatrial-transpulmonary approach, hoping to preserve global right ventricular function. We studied the short term effects on right ventricular function of either of the two approaches through a prospective randomised study, involving two comparable groups of patients operated in the same time frame.

Between June 1993 and February 1994, 40 patients having tetralogy of Fallot with comparable preoperative characteristics, were assigned randomly to each of two groups for surgical correction.

In 20 patients, correction was achieved via the transatrial-transpulmonary route. In the other 20 patients, transventricular correction was the chosen option. Six months after surgery, patients were evaluated clinically, by Doppler echocardiography, cardiac catheterisation, first pass radionuclide angiography and by 24 hours ambulatory electrocardiographic monitoring, taking note of hemodynamics, abnormalities in rhythm, and global right ventricular function.

There were no early deaths or morbidity in either group. Mean immediate postoperative ratio between peak right ventricular and systemic pressures was 0.62 ± 0.22 after transatrial and 0.70 ± 0.007 after transventricular correction. All patients were in functional class I. Six months after surgery the mean ratio between peak ventricular pressures was similar in the two groups (transatrial group: 0.37 ± 0.02, transventricular group: 0.38 ( 0.01), but significantly lower than that measured in the operating room. There were no significant arrythmias in either group. Mean right ventricular ejection fraction was nearly the same in both groups (transatrial group versus transventricular group; 44.83 ± 5.65% versus 42.37 ± 8.70%). Significant global hypokinesia of the right ventricle was documented in three patients, and mild hypokinesia in another three, undergoing repair through the transventricular route while in the group undergoing transatrial repair only one patient had mild hypokinesia.

We conclude that comparable hemodynamic results are obtained on short term follow-up after repair of tetralogy of Fallot by either the transatrial or transventricular route. Although more patients in the transventricular group were found to have global hypokinesia of the right ventricle, longer follow-up is necessary to establish the clinical relevence of these findings.
Mechanical properties of the pulmonary arteries after the arterial switch operation for complete transposition
Authors: Nakanishi Toshio , Momoi Nobuo , Kobayashi Hirohide , Nishikawa Toshio , Nakazawa Makoto , Imai Yasuharu , Momma Kazuo
Published: August 2008

This study was designed to determine, first, the stiffness of the pulmonary arteries and, second, the relationship between the stiffness of the pulmonary arteries and the success rate of balloon angioplasty in patients with complete transposition after an arterial switch operation. Indexes of pulmonary arterial wall stiffness, percent change in the radius during a cardiac cycle and the pressure elastic modulus, were calculated from the pulmonary arterial pressure and radius measured from a cineangiogram. Of the patients, 13 had no significant stenosis, while 25 had significant stenoses and, therefore, underwent balloon angioplasty. In all, we dilated 33 stenotic lesions. In the presence or absence of postoperative pulmonary stenosis, the percent change in the radius of the pulmonary trunk was significantly less than the normal value. The pressure elastic modulus of the trunk and its branches was significantly greater than normal, and increased significantly with time after the operation. In patients without pulmonary stenosis, the systolic pulmonary arterial pressure increased concomitantly with the pressure elastic modulus. Balloon dilation was successful at 17 locations and unsuccessful at 16 locations. The percent change in radius was significantly less for the failures than for the successes. Prior to 3.5 years after the operation, the pressure elastic modulus was < 400 g/cm2at most locations and balloon dilation was successful in 88%. More than 3.5 years after the operation, the pressure elastic modulus was < 400 g/cm2in 11 of 15 locations, and balloon dilation was successful in only one location. The increased stiffness of the pulmonary artery may result in increased pulmonary systolic pressure in patients without pulmonary stenosis, and result in a low success rate for balloon angioplasty in patients with pulmonary stenosis.
Interventional closure of atrial septal defects with the Amplatzer® device: first clinical experience
Authors: Bjørnstad Per G. , Masura Jozef , Thaulow Erik , Smevik Bjarne , Michelsen Svein S. , Tønnessen Tor Inge , Seem Egil , Fosse Erik
Published: August 2008

We report our initial experience in three children with percutaneous transvenous closure of significant atrial septal defects. A newly developed one-piece nitinol device is delivered through a long venous sheath of 7 French calibre under fluoroscopic and transoesophageal echocardiographic monitoring. Complete closure of all defects was verified by echocardiography on the morning after the procedure. No complication occurred. In the first two patients, the device slipped sideways into the defect. This was easily discovered and corrected. Our experience shows that the Amplatzer® device is reliable, easy to implant, and presents very clearly on transoesophageal echocardiography and fluoroscopy. This makes implantation a controlled procedure. Until release, the device can easily be retracted into the sheath at any time and removed. The results suggest that closure of an atrial septal defect with this device is feasible, rapid, and safe.
An institutional experience with the bidirectional cavopulmonary shunt: do we know enough about it?
Authors: Reddy V. Mohan , McElhinney Doff B. , Moore Phillip , Bristow James , Haas Gary S. , Hanley Frank L.
Published: August 2008

Background

The bidirectional cavopulmonary shunt has become a mainstay in the palliation of patients with a functionally single ventricle. Despite its usefulness, there are a number of concerns following its construction, including issues of timing, the development of pulmonary arteriovenous fistulas, and growth of the pulmonary arteries.

Methods

Between January 1990 and April 1996, we constructed a bidirectional cavopulmonary shunt in 123 patients. Median age at surgery was 9.4 months, and ranged from 24 days to 43 years. In 25 patients (20%), it was the first operative procedure. An auxiliary source of pulmonary blood flow was included in 72 patients (58%).

Results

Six patients (4.9%) died in the early postoperative period, and the overall early rate of failure (death or take-down) was 8.1%(n=10). By multivariate analysis, longer bypass time (p=0.012), age less than 1 month (p=0.03), and higher pulmonary vascular resistance (p = 0.043) were significant. Early reoperation was performed in 11 other patients to decrease (n=8) or increase (n=3) the amount of pulmonary blood flow. Early survivors were followed for a median of 15 months (range: 1 to 48 months), during which time 5 patients died and a Fontan circulation was completed in 30. Including early and late mortality, actuarial survival rates at 1 and 2 years were 91% and 88%, respectively. Among hospital survivors, the only significant predictor of poorer survival by Cox regression was age less than 2 months at the time of the initial cavopulmonary shunt (p=0.02). Atrioventricular valvar regurgitation was decreased after construction of the cavopulmonary shunt in 17 of the 28 patients (61%) in whom echocardiographic data were available to compare preoperative and postoperative status.

Conclusions

The bidirectional cavopulmonary shunt is a useful procedure in the early or intermediate term management of patients with a functionally univentricular heart. Much remains to be learned, nonetheless, about this unique physiologic system. The role of accessory pulmonary blood flow remains unclear, as does the use of the shunt as long-term palliation. Pulmonary arteriovenous fistulas are a serious concern, especially in young patients with heterotaxy syndrome (isomeric atrial appendages).
How classical are the clinical features of the “ostium secundum” atrial septal defect?
Authors: Tabery Stefania , Daniëls Otto
Published: August 2008

Some patients with so called “secundum” atrial septal defect within the oval fossa show a large defect on the echocardiogram, although they do not have all the classical clinical features. Until now, a large atrial septal defect was thought to be characterized by a large shunt (functional defect). Experience indicates, nonetheless, that such large defects in size (anatomical defect) are not always accompanied by large shunts. In order to assess how often the classical clinical features of an atrial septal defect exist, and to investigate whether the surgical indication for closure of the defect are anatomical or functional, we carried out a retrospective study. We evaluated the records of 161 patients, with birth dates from 1973 to 1994 (age between 0 and 21 years), so as to study the classical clinical features (history, physical examination, electrocardiogram, chest X-ray, echo-Doppler studies, cardiac catheterization, surgery). Only patients with an atrial septal defect found in isolation were included.

We discovered that the classical clinical features are often not present when there is a large defect, be it functional or anatomical. The anticipated features are present in roughly two-thirds of the patients. There is also a discrepancy between the anatomical and the functional sizes of the defect. This finding creates a fundamental problem for the future, namely the indications fot closure of atrial septal defects within the oval fossa. Further studies are needed to answer this important question
Cross-sectional echocardiographic determination of right ventricular volume and myocardial mass in children. Normal values and values in children with interatrial communication
Authors: Jin Zhen , Briedigkeit Walter , Gamillscheg Andreas , Berger Felix , Skinner Jonathan R , Golshahi Farideh , Uhlemann Frank , Lange Peter E.
Published: August 2008

Cross-sectional echocardiography was performed on 108 healthy children (7 days – 17 years old) and 55 children (6 months - 16.5 years old) with interatrial communication. Right ventricular end-diastolic volume, end-systolic volume, stroke volume, ejection fraction, muscle volume, and the ratio of muscle to cavity were calculated on the basis of outlined cavity and myocardium of an apical fourchamber view.

In the normal subjects right ventricular end-diastolic volume, end-systolic volume, stroke volume and muscle volume correlated with body surface area (end-diastolic volume: y=12.5x+7.8x2, r=0.99; end-systolic volume: y=4.8x+3.6x2, r=0.98; stroke volume: y=7.7x+4.2x2, r=0.98; muscle volume: y=14.1x+2.9x2, r=0.97), muscle/cavity ratio (0.85±0.17) and ejection fraction (58.9 ± 6.2%) were unrelated to body surface area. In the subjects with interatrial communication, the right ventricular volumes were significantly larger (p<0.001) than the normal values with a linear relationship to the ratio of pulmonary to systemic flows.

Right ventricular volumes can be determined in normal children with acceptable repeatability using a standard apical four-chamber view. The growth related normal values provide a basis for future quantitative studies.
Transcatheter embolization of abnormal intrathoracic vessels using coils in the setting of children with congenital heart disease
Authors: Pfammatter Jean Pierre , Luhmer Ingrid , Kallfelz Hans Carlo
Published: August 2008

Objective

it was the purpose of this retrospective study to assess the efficacy and the rate of complications of transcatheter embolization of abnormal intrathoracic vessels using coils in children with complex congenital heart disease.

Patients and methods

in 17 children (mean age 9.2±5.9 years) with complex congenital heart disease, occlusion by catheter intervention was attempted in 29 abnormal intrathoracic vessels: 13 aorto-pulmonary collaterals, 12 arterio-pulmonary collaterals, 2 systemic arteries supplying pulmonary sequestrations, one central venous connection and a Blalock-Taussig shunt. The mean diameter of the vessels was 4.7 ± 1.6 mm (range 2–8 mm). Steel coils with a helical diameter of three, five or eight mm were used. After selective catheterization of the vessel, they were delivered through a 5 F endhole catheter. The helical diameter of the coils was chosen in order to exceed the inner diameter of the vessel by 10–30%.

Results

as assessed by selective angiography performed 10 minutes or more after release of the coil, 27 of the 29 vessels (93%) were successfully occluded. A mean of 2.6 coils (range 1–11) were necessary for successful occlusion. Complications were encountered during 4 attempts of occlusion (14%). Of a total of 76 coils delivered, 4 coils (5%) secondarily migrated after release from the catheter, mostly to branches of the pulmonary arteries. This complication was seen predominantly in those vessels with the largest diameters. Three of the dislocated coils were left in place as they did not significantly obstruct flow of blood. One coil was retrieved by a basket catheter.

Conclusion

transcatheter embolization of abnormal intrathoracic vessels using coils in children with congenital heart disease is an effective therapy. Potential complications warrant careful evaluation of the indications for these procedures.
Three-dimensional echocardiographic assessment of right ventricular volume and function in patients with pulmonary hypertension
Authors: Apfel Howard D. , Shen Zhanqing , Boxt Lawrence M. , Barst Robyn J. , Gopal Aasha S. , Allan Lindsey D. , Gersony Welton M. , King Donald L.
Published: August 2008

Absract

The structural complexity of the right ventricle has made quantitative evaluation difficult. Conventional cross-sectional echocardiographic methods are limited by geometric assumptions and the position of the planes used for imaging. Previous reports have demonstrated accurate three-dimensional echocardiographic quantitation of the right ventricle in-vitro and in experimental animals. We adapted a previously described method for three-dimensional reconstruction of the left ventricle to compute right ventricular volume and ejection fraction in a clinical setting.

We examined 29 patients aged from 2 to 42 years with pulmonary hypertension, by three-dimensional echocardiography and resonance imaging. Correlation and agreement were calculated for volumes and ejection fractions. Three-dimensional echocardiographic reconstruction, when compared to resonance imaging, yielded r values of 0.95 and 0.93, and mean differences (bias) of 31% ± 19% and 33% ± 18%, for systolic and diastolic volumes respectively. Interobserver variability was low (12.9% and 8.0%). Ejection fraction as calculated by three-dimensional echocardiography showed close agreement with resonance images (bias=l% ±7%). Three dimensional echocardiography is now a method of measuring right ventricular ejection fraction in the clinical setting which produces results comparable to those of resonance imaging. Volume measurements correlated well for systole and diastole, but consistently underestimated values produced from resonance images.
Congenital bifid origin of right coronary artery co-existing with congenital right coronary to right ventricular fistula
Authors: Ramirez-Marroquin Samuel , Santibañez-Salgado Alfredo , Calderon-Colmenero Juan
Published: August 2008

Anomalous origin of the coronary arteries is a well-known phenomenon, but is less well reported in world literature. We present our findings in a one year old boy in whom there was bifid, or “y”, origin of the right coronary artery, co-existing with a fistula from the right coronary artery which opened into the right ventricle.
Follow-up for 30 years of a case of aortitis causing coarctation in infancy with dermatitis gangrenosa
Authors: Li Wei , Somerville Jane
Published: August 2008

A patient with aortitis presented in infancy with dermatitis gangrenosa. Coarctation of the aorta was subsequently produced by granulomatous tissue. We report 30 years of medical observation.
Parachute tricuspid valve, supravalvar tricuspid ring, and coarctation of aorta in congenitally corrected transposition
Authors: Godart François , Piot Dominique , Rey Christian
Published: August 2008

We describe an unusual case of congenitally corrected transposition in which left-sided obstructions included a supravalvar tricuspid ring, a parachute deformity of the valve itself, and coarctation of the aorta. Surgical correction was performed in 3 stages. Initially we repaired the coarctation and banded the pulmonary trunk. Then we removed the band and, finally, attempted unsuccessfully to replace the tricuspid valve. When coarctation of the aorta occurs in congenitally corrected transposition, it is always wise to search carefully for abnormalities of the tricuspid valve.
Leaking aortic aneurysm after repair of coarctation of the aorta: the significance of haemoptysis and magnetic resonance imaging
Authors: Lo S. S. S. , Kilner P. J. , Somerville J.
Published: August 2008

We discuss a patient with recoarctation of the aorta who, 4 weeks after reoperation, presented with fresh haemoptysis and was subsequently found at delayed emergency surgery to have a leaking false aortic aneurysm. The case emphasizes the significance of haemoptysis following repair of recoarctation, the difficulty of reoperation due to abnormalities of the aortic wall, and the value of serial magnetic resonance imaging in demonstrating regions of different signal intensity from haemorrhages of varying age around a leaking false aortic aneurysm.
Conversion of the modified Fontan operation to total cavopulmonary connection: a treatment for protein-losing enteropathy
Authors: Stafanger Gerd , Pettersson Gösta , Jacobsen Joes Ramsøe
Published: August 2008

We describe a patient with tricuspid atresia who, after a Fontan operation three years earlier, presented with severe protein-losing enteropathy. After conversion to a cavopulmonary connection, the symptoms regressed and substitution with albumin and immunoglobulin was no longer required.
Where's the spleen? Looking for the spleen and assessing its function in the syndromes of isomerism
Authors: Phoon Colin Kit Lun
Published: August 2008

Despite the close association of certain constellations of cardiac malformations with splenic status (right isomerism / asplenia, left isomerism / polysplenia), the association is not perfect. Given the risk of infection in the patient lacking splenic tissue, it seems prudent to assess the state of the spleen separately from the cardiovascular system, in order to decide on antimicrobial prophylaxis. A wide variety of tests is available to assess splenic function and/or anatomy. Counting pitted erythrocytes or Howell-Jolly bodies in the peripheral blood smear can be a good indicator of the lack of splenic function, although the former appears more sensitive and quantitative than the latter. Radionuclide spleen scans can provide both anatomic and functional information about the spleen, and may be a useful adjunctive test. Relatively poor spatial resolution, and interfering hepatic activity (even with spleen-specific scans), mitigate its usefulness in the young infant. The reliability of computed tomography and magnetic resonance imaging in detecting splenic tissue in the heterotaxy syndromes is unproven and perhaps only fair at best. They are probably most helpful as adjunctive tests. Ultrasonography offers excellent spatial resolution, portability, and lack of ionizing radiation, but is operator- and patient-dependent. Nonetheless, ultrasonography, coupled with an examination of the peripheral blood for Howell-Jolly bodies or pitted erythrocytes, is probably the set of tests with highest sensitivity and greatest convenience. Management strategies for the asplenic patient are also discussed.
Oral prostacyclin enhances the pulmonary vasodilatory effect of nitric oxide in children with pulmonary hypertension
Authors: Uese Kei-ichiro , Ichida Fukiko , Tsubata Shin-ichi , Hashimoto Ikuo , Hamamichi Yuji , Fukahara Kazuaki , Murakami Arata , Miyawaki Toshio
Published: August 2008

To evaluate the potential efficacy of the combined administration of inhaled nitric oxide and oral beraprost sodium in producing pulmonary vasodilation, we studied 20 patients with pulmonary hypertension during cardiac catheterization. We also evaluated some of the mechanisms of vasodilation under these circumstances by investigating the levels of cyclic GMP and AMP in the pulmonary venous blood. A significant decrease in pulmonary vascular resistance was observed after the administration of nitric oxide (−37 ± 6%, p<0.01), and more intense decrease was observed after the combined administration of nitric oxide and beraprost sodium (−46 ± 6%, p<0.05). In addition, the ratio of pulmonary-to-systemic resistance also decreased to a greater extent with combined administration than with nitric oxide alone (−34 ± 6% vs −42 ± 7%, p<0.05). Conversely, systemic vascular resistance showed no change, neither after loading with nitric oxide nor after combined administration. Although equivalent increases in levels of cyclic GMP were observed after inhalation of nitric and the combined administration (mean 78% vs 65%), greater increases in levels of cyclic AMP were observed with the combined use (mean 15% vs 69%). Combined treatment with nitric oxide and beraprost sodium may prove markedly beneficial in patients with primary and postoperative pulmonary hypertension who do not respond adequately to inhalation of nitric oxide alone or conventional modes of treatment.
The role of angiogenesis in the development of pulmonary arteriovenous malformations in children after cavopulmonary anastomosis
Authors: Marshall Blair , Duncan Brian W. , Jonas Richard A.
Published: August 2008

Pulmonary arteriovenous malformations are a frequent cause of progressive cyanosis after construction of a cavopulmonary anastomosis. Their formation complicates the management of children with single ventricle physiology after a bidirectional Glenn shunt or the Kawashima procedure. The key role of the liver in this phenomenon is suggested by the observation that providing modifications of the Fontan procedure which permit hepatic venous effluent to reach the pulmonary arterial circulation limit further development of the malformations. In addition, it is known that patients with end-stage hepatic failure develop pulmonary arteriovenous malformations that diminish after liver transplantation. We have begun purification of a factor derived from hepatocyte-conditioned media that is inhibitory for the proliferation of cultured endothelial cells. This substance is heat sensitive, and binds avidly to a copper-containing chromatography column. These clinical observations, and this preliminary experimental work, support the concept that hepatic-derived angiogenic factors may play a role in the development of pulmonary arteriovenous malformations after construction of cavopulmonary anastomoses.
Venous obstruction due to insertion of permanent transvenous pacemaker electrodes in children
Authors: Çeliker Alpay , Tokel Kürsad , Balkanci Ferhun , Çekirge Saruhan , Oto Ali , Özme Şencan
Published: August 2008

Transvenous placement of pacemaker leads is the recommended method for permanent pacemaking in children. We evaluated 11 children with transvenous ventricular pacemaker leads by digital subtraction angiography in order to determine the incidence of venous obstruction. The ages at the implantation ranged from 4.5 to 11.2 years with a mean of 8.05±2.17 and a median of 7.9 years. The interval from implantation to the time of procedure ranged from 12 to 64 months, with a mean of 37.4±19.2 and a median of 45 months. Complete occlusion of the subclavian vein was demonstrated in two patients. We also showed significant stenosis of the venous system in five further patients. Of the 7 patients, 5 had an extensive venous collateral circulation. We did not observe obstruction of the superior caval vein, or superior caval venous syndrome. Although we did not demonstrate this syndrome, we propose future regular evaluation of all our children undergoing transvenous pacemaking. This point may be important when second or third leads are implanted.
Progression of congenital aortic stenosis in children beyond infancy: assessment using Doppler echocardiography
Authors: Goel Punit , Kumar Krishan , Kothari S.S. , Saxena Anita , Wasir Harbans S.
Published: August 2008

Case records of 67 children who presented beyond infancy (57 male, 10 female) with congenital valvar aortic stenosis were reviewed to assess progression utilizing the Doppler derived peak gradient as the index of severity. Age at presentation ranged from 1–13 years, and mean follow-up was 67 ± 29 months (range 12–142 months). The patients were divided into three groups depending on the rate of progression. Those with the most rapid rate of progression were significantly older when aortic stenosis was detected and at the time of presentation compared to the other two groups, who progressed minimally or not at all (p < 0.002 and p < 0.01 respectively). There was no significant correlation between the progression of stenosis and other clinical or Doppler echocardiographic variables.
Cerebral abscess in children with cyanotic congenital heart disease
Authors: Baysal Kemal , Öztürk Fadil , Uysal Serap , Sancak Recep , Gürses Nuran
Published: August 2008

The cerebral abscess still occurs in children with cyanotic congenital heart disease. Mortality and morbidity have not yet been reduced in spite of new antibiotics and surgical techniques. We describe our experience with 185 patients having cyanotic congenital heart disease (117 with tetralogy of Fallot, 23 with complete transposition, 9 with tricuspid atresia, 4 with total abnormal venous return, 10 with double-inlet ventricle, 15 with double-outlet right ventricle and 7 with common arterial trunk) who have been followed regularly in our department. Their ages ranged between 3 years and 15 years. During the period of follow-up, cerebral abscess developed in 7 cases. Six of them had tetralogy of Fallot and one had complete transposition. One patient died before surgery could be performed. Operation was carried out in 6 patients and one died in the early postoperative stage. The others were as healthy as could be expected. Alpha-hemolytic streptococcus was isolated from the abscess in one patient, and Enterobacter in another. The abscesses were localized in the occipito-parietal region in 2 cases, the fronto-parietal region in 2 cases, and the parietal, temporal and occipital regions in one case each. Physical examination was normal apart from fever in 5 patients and, in these patients, the cause of fever could not be explained. Neurologic evaluations were all within normal range. If fever occurs unexpectedly in a patient with cyanotic congenital heart disease, therefore, it is important to rule out the possibility of a cerebral abscess.
Infectious endocarditis in an analysis of fifty-five episodes over a ten-year period
Authors: Wong Tracy , Fowler Rodney S. , Freedom Robert , Johnston Douglas H.
Published: August 2008

Mortality from infectious endocarditis in the general paediatric population had decreased significantly over the last two decades. This is attributable to the increased awareness of the health care team in recognizing the early signs of this once fatal disease such that early antibiotic intervention is possible. Two groups, however, remain at high risk for infectious endocarditis. Advances in cardiac surgery have dramatically improved the prognosis for survival in many children with otherwise fatal congenital heart diseases. This group of children, along with the seriously ill who have central venous catheters in place, are currently at the highest risk for development of infectious endocarditis.1-5
Clinical profile and prognosis of hypertrophic cardiomyopathy when first diagnosed in infancy as opposed to childhood
Authors: Comparato Calogero , Pipitone Salvatore , Sperandeo Velio , Mongiovì Maurizio
Published: August 2008

Only small groups of children, and especially infants, with hypertrophic cardiomyopathy have been analysed thus far. The clinical features, and the evolution, of this potentially heterogeneous disease, therefore, remain to be clarified. We describe the clinical profile of 35 patients with hypertrophic cardiomyopathy, 18 being younger than 1 year when first seen. Electrocardiography, chest radiography and echocardiography (m-mode, cross-sectional and color-Doppler) were performed in all patients. Ambulatory electrocardiographic monitoring was performed in 20 patients, and cardiac catheterization in 5 patients. The duration of follow-up was 6.6 years, and ranged from 1 month to 15 years. First-degree relatives of 9 infants and 13 children underwent complete echocardiographic evaluation.

Inheritance, along with presence of congestive heart failure, cyanosis, cardiac enlargement and right ventricular hypertrophy proved to be different in infants who presented with hypertrophic cardiomyiopa-thy as compared with those presenting in childhood. In contrast, echocardiography showed that the distribution of ventricular hypertrophy was similar in the two groups. Intractable congestive heart failure, which was the commonest cause of death, was an ominous prognostic sign in infants. Older children, however, tended to die suddenly. Spontaneous resolution of severe ventricular hypertrophy occurred in four infants of non-diabetic mothers within their first 6 months of age. We conclude that hypertrophic cardiomyopathy may represent a constellation of disease entities. The observed spontaneous resolution in 4 infants may represent a new form of the syndrome which carries a good prognosis.
Comparison of right ventricular volumes by transthoracic and transesophageal echocardiography in patients with right ventricular volume overload
Authors: Gamillscheg Andreas , Jin Zhen , Skinner Jonathan , Schulze-Neick Ingram , Nürnberg Jan-Hendrik , Berger Felix , Uhlemann Frank , Lange Peter E.
Published: August 2008

Assessment of right ventricular volume and function is important in most congenital heart diseases before and after corrective or palliative surgery. Since transthoracic echocardiography is often substituted by transesophageal echocardiography in the perioperative setting, it is useful to compare transesophageal echocardiography with transthoracic echocardiography as performed preoperatively. We compared right ventricular volumes as calculated using these two methods from a four-chamber view in 21 children and adults with atrial septal defect. For right ventricular end-diastolic volumes of less than 70 ml, and end-systolic volumes of less than 40 ml, a close correlation was found between the techniques (r=0.99 and r=0.91, respectively), with a small degree of underestimation by transesophageal echocardio­graphy. For values larger than 70 nil and 40 ml, respectively, correlation decreased (r=0.41 for end-diastolic volumes and r=0.48 for end-systolic volumes) and underestimation of volume by transesophageal echocardiography increased. Underestimation of right ventricular end-diastolic volumes increased with increasing body surface area (r=0.74), and with progressive right ventricular enlargement (r=0.63). In patients with a body surface area of more than 1m2, the largest end-diastolic right ventricular length determined by transthoracic echocardiography was significantly longer than that derived by transesophageal echocardiography (p<0.001), whereas in smaller patients there was no significant difference between the two methods (p>0.1). If right ventricular volumes determined by transthoracic echocardiography using a four-chamber view are substituted by those obtained with transesophageal echocardiography in serial haemodynamic evaluation of patients with atrial septal defect, different correlation equations and, consequently, a different degree of underestimation by transesophageal echocardiography must be considered for large and small volumes. This increasing underestimation of larger right ventricular volumes seems to be based on foreshortening of the long cross-sectional axis of the right ventricle as seen in the transesophageal four-chamber view.
Anomalous origin of the right coronary artery from the pulmonary trunk, echocardiographic and colour Doppler features
Authors: Barlow Amanda J. , Sandor George G.S. , Le Blanc Jacques G.
Published: August 2008

Anomalous origin of the right coronary artery from the pulmonary trunk is a rare congenital anomaly. Diagnosis is usually made at autopsy or incidentally in asymptomatic adults, but cardiopul-monary arrest and sudden death have been reported. The angiographic findings have been well described but the echocardiographic findings are less well documented.

This report describes the echo and colour Doppler findings in a 3-year-old child with an anomalous right coronary artery arising from the pulmonary trunk, as well as observations on myocardial perfusion demonstrated by nuclear angiography.
Neonatal hypertrophic cardiomyopathy with duct-independent systemic blood flow
Authors: Suda Kenji , Horton Darlene P. , Silverman Norman H.
Published: August 2008

We describe a newborn who had echocardiogaphic features of severe hypertrophic cardiomyopathy and duct-dependent systemic blood flow. Blood flow to the upper body could be maintained only by prostaglandin El, as in the case of hypoplastic left heart syndrome. Forward flow was gradually established from the left ventricle, and the patient weaned from prostaglandin El. Although the patient's mother had poorly controlled diabetes, the patient at 24 months of age shows residual but lessened ventricular hypertrophy and increased cavity size.
Discordant atrioventricular and ventriculoarterial connections with partially unguarded left-sided atrioventricular orifice and aortic atresia
Authors: Freund Matthias , Magener Achim , Schmidt Klaus G.
Published: August 2008

Congenitally unguarded morphologically tricuspid valvar orifice is a rare abnormality, usually associated with pulmonary atresia. We describe such a case found in association with discordant atrioventricular and ventriculoarterial connections in which the aortic valve was atretic. Presentation was with respiratory distress due to severe cardiac failure after birth. Echocardiography demonstrated a dilated systemic ventricle with poor function, left atrial enlargement, and aortic atresia. The clinical findings were confirmed at autopsy.
Infantile scimitar syndrome with severe pulmonary hypertension: successful treatment with coil embolization of the systemic arterial supply to the sequestered lung
Authors: Pfammatter Jean-Pierre , Luhmer Ingrid , Kallfelz Hans Carlo
Published: August 2008

Scimitar syndrome is a rare cardiopulmonary malformation. Presentation in infancy is usually associated with pulmonary hypertension and severe symptoms. We discuss treatment of such an infant. Two abnormal systemic vessels supplying the sequestered lower part of the right lung were embolized using catheter-inserted coils. The patent arterial duct was surgically ligated. These procedures resulted in a significant reduction of the shunt and the level of pulmonary hypertension, as well as in an impresssive improvement of symptoms.
Balloon angioplasty for isolated coarctation of the aorta with mirror image right aortic arch in an infant
Authors: Tsubata Shinichi , Ichida Fukiko , Miyawaki Toshio
Published: August 2008

We report the first infant case with coarctation of the aorta and mirror image right aortic arch, who presented with severe myocardial dysfunction and underwent successful balloon angioplasty for relief of coarctation, avoiding anesthetic and surgical risk. A follow-up cardiac cathetelization revealed no residual pressure gradient and continued angiographic improvement with excellent restoration in left ventricular function.
Left ventricular fibroma in two children
Authors: Joo Chan Uhng , Kim Kong Soo , Yoon Hyang Suck
Published: August 2008

Primary cardiac tumors are rare in infancy and childhood. We describe the features of large intramural ventricular cardiac fibromas discovered in two children. In the first, a left ventricular apical tumor was discovered by means of echocardiography and left ventriculography in a symptomless 8-year-old boy. The intramural fibroma was successfully resected. In the other case, an 8-month-old female infant with severe congestive heart failure, sudden ventricular tachycardia and fibrillation occurred during left ventriculography. At necropsy a tumor was seen bulging in the anterior wall of the left ventricle and in the ventricular septum. Histology showed the tumor to be a fibroma.
Congenitally corrected transposition presenting at 24 years: errors of management
Authors: Gerlis L. M. , Egdell R. , Somerville Jane
Published: August 2008

A 24-year-old female without previous symptoms or known heart disease consulted a general practitioner with dyspnoea which had slowly worsened over a period of two years. She was treated for asthma, without investigations, until she presented with rapid atrial fibrillation, chest pain and pulmonary oedema necessitating admission to hospital, where she was found to have “mitral” regur-gitation (of the left-sided atrioventricular valve) and congenitally corrected transposition. At operation the grossly regurgitant left-sided morphologically tricuspid valve was replaced by a “top-hat” inverted aortic valve homograft. The procedure resulted in intermittent heart block, needing a pacemaker, and continued myocardial failure which progressed with new right-sided valvar regurgitation. She died a few months later. Post mortem examination confirmed the double discordance, and showed a fistula around the tricuspid valvar prosthesis, left atrial thrombus and changes in the mitral valve. There was also an unsuspected area of old myocardial ischaemia, associated with severe hypoplasia of the circumflex branch of the right-sided coronary artery. This was considered probably to have been of embolic origin.
Surgical controversies: coarctation
Authors: Hamilton J. R. L.
Published: August 2008

Fifty one years have passed since grossand Crafoord independently reported successful repair of aortic coarctation. One could be forgiven for assuming that all the surgical controversies have now been settled. This is far from the case, as the numerous publications (frequently with opposing view points) related to timing, technique and complications bear testament. Perhaps the passage of the golden anniversary of this operation should stimulate a degree of reflection among surgeons and cardiologists.
Pulsed Doppler echocardiographic assessment of patterns of venous flow after the modified Fontan operation: potential clinical implications
Authors: Kaulitz Renate , Luhmer Ingrid , Kallfelz Hans Carlo
Published: August 2008

To assess the effect of a modified Fontan operation on systemic venous blood flow and the hepatic circulation, we compared 11 patients having an atriopulmonary connection and 35 with total cavopulmonary anastomosis. The Doppler echocardiographic study of the caval venous, hepatic venous and portal venous flow was performed so as to calculate the pulsatility ratio and the variation of flow with respiration. All patients had undergone cardiac catheterization. In addition, we included specific laboratory investigations to assess function of various organs.

Significantly lower maximum velocities of flow at inspiration (0.31±0.12 rn/sec vs 0.45±0.14 m/sec) and expiration (0.23±0.09 rn/sec vs 0.32±0.11 m/sec), less pulsatility (0.43 vs 0.16) and a lower ratio of systolic to diastolic velocity (1.22 vs 1.85) were found in the patients having a cavopulmonary as compared to an atriopulmonary anastomosis. Peak velocities of hepatic venous flow during inspiration and expiration were significantly lower in those with a cavopulmonary anastornosis (p=O.OOl and p<O.OOl, respectively). In these patients, forward flow was extremely dependent on respiration, with decrease or cessation of antegrade flow during expiration in 22 patients. The velocity of portal venous flow was also significantly lower in these patients, although the pulsatility ratio did not differ significantly between the groups (0.5 ± 0.21 and 0.57 ± 0.23, respectively). The ratio of inspiratory and expiratory velocities showed no significant difference between the groups, nor was there any correlation between the pulsatility ratio of the venous vessels or the ratio of peak flow velocities during expiration and the mean systemic venous/right atrial pressure on postoperative cardiac catheterization. Hypoproteinemia was found in 8 patients after total cavopulmonary anastomosis; 9 of 10 patients with protein C deficiency belonged to this group.

The dependence of hepatic venous flow on respiration in the presence of a chronically elevated systemic venous pressure in patients after the total cavopulmonary anastornosis may influence hepatic function in the postoperative period.
Hemolysis associated with coil occlusion of the arterial duct
Authors: Pedra Carlos A. C. , Pedra Simone F. , Font Valmir F.
Published: August 2008

Severe mechanical hemolysis ocurred in a 22 month old girl after placement of a 38-5-10 coil in the arterial duct. Shé had previously undergone percutaneous closure using the Rashkind technique 14 months before insertion of the coil, but remained with a moderate residual shunt. Surgical removal of the devices and division of the duct were required to abolish the hemolysis.
Mobility of the flap valve of the primary atrial septum in the developing human fetus
Authors: Firpo Cora , Zielinsky Paulo
Published: August 2008

It has already been suggested that redundancy of the primary atrial septum could be associated with primary fetal arrhythmias. There are few reports, however, concerning the mobility of the primary septum and its behavior during fetal life. In this study, fifteen fetuses without anatomic heart disease were serially evaluated by fetal echocardiography. The time interval between three separate examinations was at least two weeks. A redundancy index was calculated as the ratio between the maximum excursion of the flap valve of the septum and the maximal left atrium as seen in a four chamber view. The indexes observed in the three examinations were: 0.55 ± 0.15, 0.48 ± 0.13 and 0.41 ± 0.13, respectively. Analysis of variance for normal distribution and Tukey's test were performed. A significant decrease (p < 0.05) in the redundancy index was observed with increasing gestational age. We postulate that this finding may be related to the decrease in frequency of premature atrial beats throughout fetal life.
Transoesophageal echocardiographic assessment of obstruction to the pulmonary venous pathway in children with Mustard or Senning repair
Authors: Hashmi Aijaz , Hosking Martin , Teixeira Otto , Cornel Gary , Duncan Walter
Published: August 2008

The morphology and mechanism of obstruction to the pulmonary venous pathway in patients following either Mustard or Senning repair of complete transposition was assessed using transoesophageal echocardiography. Seven patients underwent catheterization and complete transoesophageal study in both transverse and longitudinal planes, followed by balloon dilation of the obstructed venous pathway in five of seven under transoesophageal echocardiography guidance. A complete scan of both systemic and venous pathway was obtained in all patients. Four patients with a Mustard repair were found to have a ‘tubular’ baffle, with stenosis resulting from a discrete wedge of tissue arising from the atrial free wall in association with fibrous adhesions to the baffle. In the three patients with a Senning repair the intra-atrial baffle showed a characteristic ‘peaked’ appearance, with stenosis of the venous pathway stenosis related directly to con tracture of the patch used to augment the atrial free wall. The mechanism of obstruction appears to be inherent to the different surgical techniques. Indwelling transoesophageal echocardiography provided immediate haemodynamic and morphologic assessment of the efficacy of dilation of the obstructed venous pathway.
Compression of the pulmonary veins by the descending aorta in patients corrected surgically by the Fontan procedure
Authors: Kawahira Youichi , Kadoba Keishi , Matsuda Hikaru
Published: August 2008

We describe 4 patients in whom the pulmonary veins were compressed between the descend ing aorta and the atrial part of the heart in consequence of an abnormal distribution of the pulmonary blood flow after the Fontan procedure.
Repair of the mitral valve because of pure rheumatic mitral valvar incompetence in the young
Authors: Al Jubair Khalid A. , Jaralla Abdullah , Fadala Mohsen , Bukhari Emad , Al Faraidi Yahya , Al Qethami Huwaida , Al Fagih Mohamed R.
Published: August 2008

Between January 1985 and April 1994, 42 children aged between 7 and 14 years (mean 11.3 years) underwent repair of severely incompetent rheumatic mitral valves with no accompanying mitral stenosis. In 19 patients the tricuspid valve was severely incompetent, while 8 patients had severe aortic incompetence. Overall, the repair incorporated shortening of elongated tendinous cords and insertion of a Duran or Carpentier prosthetic ring. The repair was the sole procedure in 15 patients, whilst 19 patients also had a De Vega tricuspid valvar annuloplasty and 8 had repair or replacement of the aortic valve. There were no hospital deaths. Postoperative transthoracic echocardiographic studies revealed trivial residual mitral valvar regurgitation in 6 patients, mild regurgitation in 18, moderate in 15, and regurgitation severe enough to warrant replacement of the mitral valve in 3 patients. The mean follow-up period was 37 months (maximum 120 months, minimum 1 month). 0139 patients followed-up, 28 attended for more than 5 years. Of these, 7 underwent replacement of the mitral valve for severe regurgitation within 4 years of the repair. Severe mitral regurgitation in 3 patients was controlled by medical therapy. One was eventu ally lost to follow-up, and one patient died of causes unrelated to surgery. The remaining 16 patients had absent to moderate mitral valvar regurgitation. These results contrast with 10 repairs of congenital mitral incompetence, where no patients required re-operation in the immediate 5 years period of follow-up. Every effort should be made in children with rheumatic mitral incompetence to preserve the natural valve by con servative repair, despite the fact that repair of the incompetent rheumatic mitral valve is not so durable as repair of congenitally incompetent valves.