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Introduction: Tetralogy of Fallot is characterized by a non-restrictive malalignment ventricular septal defect (VSD), an overriding aorta and right ventricular outflow tract obstruction resulting from anterior deviation of the infundibular septum. Due to the large VSD, systolic pressures are equal in both ventricles. In rare cases, redundant tricuspid valve tissue may prolapse into the VSD resulting in restriction of the defect size and causing suprasystemic pressure in the right ventricle. Subaortic obstruction by prolapse of the redundant tricuspid tissue into the left ventricular outflow tract, although theoretically possible in this situation, has not been described yet in the literature., Case Report: We report on a newborn with tetralogy of Fallot presenting with cyanosis and severe arterial hypotension a few hours after birth. Echocardiography revealed redundant hammock-like accessory tricuspid valve tissue almost completely occluding the originally large VSD. Suprasystemic pressure in the right ventricle resulted in protrusion of tricuspid valve tissue across the VSD and thereby caused severe left ventricular outflow tract obstruction. Emergency pulmonary balloon valvuloplasty performed for decompression of the right ventricle, reduced prolapse of tricuspid tissue into the left ventricular outflow tract and resulted in significant improvement of pulmonary and systemic blood flow., Conclusion: In tetralogy of Fallot, accessory tricuspid valve tissue may obstruct the VSD as well as the left ventricular outflow tract resulting in a life threatening condition in newborns shortly after birth.

We report three patients with partial atrioventricular septal defect who presented in the neonatal period with excessive left to right shunting and progressive pulmonary hypertension. Successful biventricular repair was accomplished despite the fact that left ventricular area and left sided structures did not meet the criteria established previously for biventricular management in children with critical aortic stenosis or complete atrioventricular septal defect. Indication for biventricular management was based on the fact that none of our patients had morphological mitral or aortic stenosis and that the hypoplastic left ventricle proved capable to maintain the systemic circulation following closure of the arterial duct. Fenestrated closure of the atrial septum and an individualized approach regarding the closure of the cleft in the left sided atrioventricular valve appear to be important surgical options to reduce postoperative left atrial pressure and to allow the ventricles to adapt to the new loading conditions.

Purpose: Total anomalous pulmonary venous connection (TAPVC) is a rare congenital cardiovascular malformation that is characterized by an absent connection of the pulmonary veins to the left atrium. Echocardiographic differentiation of TAPVC and persistent pulmonary hypertension of the newborn (PPHN) can be a great diagnostic challenge. The aim of our study was the assessment of a systematic echocardiographic approach to evaluate the feasibility and reliability of noninvasive diagnosis of TAPVC., Methods: Between January 1995 and January 2005, 15 consecutive patients with isolated TAPVC were diagnosed in our institution. 5 patients had supracardiac type, 5 cardiac type, 3 infracardiac and 2 had mixed type of TAPVC. Since 2001 preoperative diagnosis was performed exclusively by echocardiography (8/15 patients)., Results: TAPVC could be diagnosed correctly in all cases using a systematic echocardiographic approach with careful investigation of the proximal cardiac veins. Infracardiac type ofTAPVC was diagnosed using systematic evaluation of the liver with visualization of a pathological vessel with venous flow directed away from the heart. In all cases the echocardiographic findings were confirmed during surgery., Conclusion: Echocardiography with Doppler and color Doppler sonography is an effective method for noninvasive diagnosis of TAPVC. The presented systematic echocardiographic approach permits reliable noninvasive differentiation of TAPVC and PPHN.

We report a simple valve repair for severe pulmonary incompetence in a 25-year-old patient. The patient had been operated on twice before for ventricular septal defect and coarctation of the aorta. The first operation consisted of pulmonary artery banding and coarctectomy and end-to-end anastomosis at 4 months, followed by debanding and transinfundibular ventricular septal defect closure at 6 years of age. Massive pulmonary incompetence was due to destruction of one valve cusp with the right ventricular outflow tract and pulmonary artery dilated secondarily. Repair consisted of pulmonary valve bicuspidization and right ventricular remodelling.

Unlabelled: PURPOSE/MATERIALS AND METHODS: The aorto-pulmonary window is a rare congenital cardiac malformation consisting of a connection between the ascending aorta and the main pulmonary artery. Based on 5 consecutive patients who were treated in our tertiary referral centre between 7/2000 and 11/2005, we report the echocardiographic features as well the limitations of this diagnostic method in the assessment of this rare malformation., Results: Dilation of the left atrium and ventricle due to the large left-to-right-shunt was the characteristic echocardiographic feature in all patients with aorto-pulmonary window. In all patients we were able to visualise the window by 2D-echocardiography. Pulsed- and continuous-wave Doppler showed diastolic negative flow in the descending aorta and in systemic arteries due to left-to-right shunting in diastole via the aorto-pulmonary window. All 5 patients had significant associated cardiovascular malformations. They were accurately diagnosed by echocardiography. Coronary anomalies could be excluded noninvasively in all patients., Conclusion: Echocardiography is the method of choice for diagnosis of aortopulmonary window. Detection of aortopulmonary window, however, requires a systematic and careful investigation of all cardiovascular anatomic details, since this anomaly is frequently associated with substantial additional cardiac anomalies, which would by themselves be sufficient to explain the haemodynamic abnormalities of the patient. Cardiac catheterisation is required only in cases with inadequate information about coronary artery anatomy or in older patients with pulmonary hypertension, to determine the patient's suitability for surgical correction.

Objectives: Thrombotic events have been reported as a major cause of morbidity after the Fontan procedure. There is no consensus concerning the postoperative mode and duration of anticoagulation prophylaxis. In a retrospective study, we evaluated the results of a prophylactic regimen on the basis of the surgical technique, potentially predisposing risk factors, and specific sequelae., Methods: We evaluated 142 surviving patients after total cavopulmonary anastomosis (mean follow-up was 91.1 +/- 43.9 months). Prophylactic antithrombotic treatment was initiated in 86 patients with partial prosthetic venous pathway with acetylsalicylic acid; 45 patients with complete autologous tissue venous pathway or partial prosthetic venous pathway received no anticoagulation, and 11 patients received warfarin sodium (Coumadin). During long-term follow-up, 22 patients (12 after acetylsalicylic acid medication) crossed over to warfarin., Results: Thrombotic events occurred in 10 patients (7%), with systemic venous thrombus formation in 8 (5.6%), stroke in 2 (1.4%), and a peak incidence during the first postoperative year. Eight of 10 patients were receiving heparin therapy mainly for prolonged postoperative immobilization. During follow-up, none of the 74 patients receiving acetylsalicylic acid and 1 of 40 patients without medication presented with thrombus formation. Under warfarin medication, 1 of 28 patients had an asymptomatic thrombus. Expected freedom from a thromboembolic event was 92% at 5 years and 79% at 10 years. There was no association with coagulation factor abnormalities. Protein-losing enteropathy was present in 4 of 10 patients., Conclusion: A prophylactic anticoagulation strategy that considers the surgical technique and potential predisposing circumstances proved effective in the prevention of late thrombotic complications after total cavopulmonary anastomosis. There is no need for routine anticoagulation during long-term follow-up after Fontan-type surgery in pediatric patients.

Objective: To assess the sensitivity and specificity of echocardiography in the detection of anomalies of the subclavian artery (cervical origin, aberrant origin from the descending aorta or isolation) in patients with monosomy 22q11., Method: From 1/1998 until 03/2002 we examined 57 patients with conotruncal cardiac malformations. 30 patients had pulmonary atresia and ventricular septal defect (53 %), 13 interrupted aortic arch (23 %), 9 tetralogy of Fallot (16 %) and 5 common truncus arteriosus (9 %). Echocardiographic examination included identification of the laterality of the aortic arch as well as examination of the origin of the brachiocephalic vessels. The results were compared with angiographic and intraoperative findings. Median age at echocardiographic investigation was 74 days (range 1 d - 33.4 yrs.)., Results: Laterality of the aortic arch was assessed correctly in all patients. 20/57 patients had a right-sided aortic arch (35 %). Echocardiography detected all anomalies of the subclavian artery (14/57 patients, 25 %, sensitivity 100 %) and excluded these anomalies correctly in 43 patients (specificity 100 %). Exact classification of the anomaly of the subclavian artery was possible in 6/6 patients with a cervical origin of the artery and in 7/8 patients with aberrant origin from the descending aorta. Monosomy 22q11 was diagnosed in 21 patients (37 %)., Conclusion: Echocardiography achieves a high sensitivity in the detection of anomalies of the subclavian artery. The diagnosis of cervical origin of the artery, in particular, can be easily established. As this anomaly appears to be a specific marker for monosomy 22q11, echocardiography facilitates reliable identification of these patients in clinical practice.

Atrial isomerism and visceral heterotaxy describe complex pathoanatomic findings with defects in the determination of lateralization. Differentiation of right and left atrial isomerism was based on the anatomy of the atrial appendages and bronchial tree and often associated with asplenia in right atrial isomerism and polysplenia in left atrial isomerism. In these pa tients complex cardiac anomalies determine clinical symptoms and diagnostic procedures. At primary diagnosis in the neonatal period, systemic venous and pulmonary venous connections and intracardiac anatomy are identified using the sequential segmental approach. Therapeutic decisions and palliative procedures can usually be based on these initial echocardiographic findings. As many of the patients were found to have a functionally univentricular heart, surgical procedures following the Fontan principle (lateral tunnel or extracardiac conduit procedure) were introduced in patients with heterotaxy syndrome and successfully performed. Early survival and long-term outcome vary depending on associated cardiac and systemic and pulmonary venous anomalies. The therapeutic options and mid-term results in patients with heterotaxy syndrome undergoing the Fontan procedure are reported.

Unlabelled: A variety of cardiac defects, encompassing truncus arteriosus, tetralogy of Fallot, pulmonary atresia with ventricular septal defect and interrupted aortic arch, are generally summarised as conotruncal malformations. Patients with these cardiac defects were frequently found to have a common microdeletion on chromosome 22, the so-called monosomy 22q11.2. The aim of our study was to determine whether the laterality of the aortic arch or the presence of subclavian artery anomalies (SAA) represent markers for monosomy 22q11.2 in these patients. 170 patients with these cardiac anomalies were recruited at presentation in the paediatric cardiology units of two tertiary referral centres from 1994 until 2003. Of the 170 children and young adults, 33 had interrupted aortic arch, 35 tetralogy of Fallot, 31 truncus arteriosus communis and 71 pulmonary atresia with ventricular septal defect. All were screened for monosomy 22q11.2 and the results were correlated with the laterality of the aortic arch and the presence of SAA contralateral to the aortic arch (aberrant origin from the descending aorta, isolation, distal ductal origin from the pulmonary artery and cervical origin of the right subclavian artery). Monosomy 22q11.2 was present in 59/170 patients (35%). A left aortic arch (LAA) was found in 118 (69%), a right aortic arch (RAA) in 52 (31%) patients. Almost 50% of the patients with RAA (46%), but only 30% of the patients with LAA had monosomy 22q11.2 ( P=0.054). A total of 47 patients (28%) had an anomaly of the subclavian artery, 81% of whom had monosomy 22q11.2. This deletion was found in decreasing percentage in patients with LAA+SAA (85%) >RAA+SAA (75%) >RAA without SAA (28%) >LAA without SAA (13%)., Conclusion: In patients with conotruncal malformations, anomalies of the subclavian arteries are the most important anatomical marker for the presence of monosomy 22q11.2, independent of the laterality of the aortic arch. Therefore, we recommend cytogenetic testing for this microdeletion in all patients with subclavian artery anomalies and conotruncal malformations.

Aim/method: The aorto-left ventricular tunnel (ALVT) is a rare congenital cardiac malformation with clinical findings of severe aortic insufficiency. We report the echocardiographic differentiation of different anatomical variants of ALVT in two infants., Results: Echocardiography in both patients demonstrated severe enlargement of the ascending aorta and the left ventricle. In the first patient the ALVT originated from the ascending aorta above the right coronary sinus and entered the left ventricle just below the aortic valve. In the second patient the ALVT originated above the left coronary sinus and took a lateral course to the left ventricle. Colour-Doppler-sonography in both patients confirmed a systolic-diastolic flow across the tunnel. Many patients have associated cardiac defects. Exact determination of the morphology of the aortic valve and coronary arteries is mandatory for surgical repair. Postoperative follow-up studies focus on the function of the aortic valve and the left ventricle., Conclusion: Differentiation of different anatomical variants of ALVT is possible trough echocardiography. Cardiac catheterization is required only in cases with inadequate information about coronary artery anatomy.

Anomalous origin of one pulmonary artery from the ascending aorta is a rare anomaly which is almost always associated with hypertension in the contralateral pulmonary artery originating from the right ventricle. We report echocardiographic evaluation of an infant with tetralogy of Fallot and aortic origin of the right pulmonary artery. Since all relevant information regarding anatomy and hemodynamics could be obtained by echocardiography, cardiac catheterization and angiography were unnecessary. Surgical correction including transatrial patch closure of the ventricular septal defect, resection of the infundibular stenosis and direct reimplantation of the right pulmonary artery was performed successfully at the age of 7 weeks. The postoperative course was uneventful and persistent pulmonary hypertension was excluded by echocardiography. Our case shows that complete echocardiographic evaluation is possible in infants with this complex cardiac malformation. We recommend reserving cardiac catheterization for those patients with echocardiographic evidence of significant elevation of pulmonary vascular resistance. In order to avoid irreversible pulmonary vascular changes early surgical correction within the first 3 months of life should be performed in all children with aortic origin of a pulmonary artery including those with associated tetralogy of Fallot.

Background: The purpose of this study was to determine the type and incidence of hemodynamic and electrophysiological abnormalities requiring surgical or catheter-based interventions in a single-center long-term experience., Methods: Eighty-eight patients with a follow-up of at least 5 years (mean follow-up, 9.6 +/- 2.6 years) after Fontan-type procedures were included. All patients had undergone cardiac catheterization either as part of the regular postoperative protocol or because of symptomatic atrial tachycardia or increasing cyanosis., Results: Freedom from reoperation for up to 5 years was documented for 82% of patients and decreased to 76% after 8 years. Late reoperations included conversion of an atriopulmonary anastomosis to a total cavopulmonary anastomosis in 2 patients with atrial dysrhythmia and implantation of an extracardiac conduit in 1 patient with left atrial isomerism and intrapulmonary arteriovenous malformations after a Kawashima operation. Decline in sinus node function with symptomatic bradycardia required pacemaker therapy in 10 patients (11%). Transcatheter interventions included fenestration occlusion in 5 of the 11 patients with initial baffle fenestration. In 6 of 17 patients with aortopulmonary collaterals, coil occlusion was indicated to reduce future systemic ventricular volume load. Various systemic venous collaterals were documented in 11 patients and required coil occlusion in 2. One patient with symptomatic protein-losing enteropathy underwent transcatheter fenestration creation without sustained relief of symptoms. Freedom from transcatheter interventions decreased from 94% to 82% after 5 and 10 years, respectively., Conclusions: During long-term follow-up, reoperations are rare and mainly involve Fontan conversion to either a lateral-tunnel or extracardiac-conduit procedure. Detailed angiographic evaluation on a routine basis allows identification of the vascular sites of origin of aortopulmonary collateral vessels and systemic venous collaterals potentially developing during long-term follow-up. Transcatheter interventions including fenestration occlusion and occlusion of venous collaterals and aortopulmonary collaterals were performed to maintain and improve the Fontan circulation in clinically symptomatic and asymptomatic patients. During long-term follow-up after Fontan-type operations, a regular postoperative cardiac catheterization protocol is recommended.

Data on mid- and long-term follow-up for the recent devices for closure of secundum-type atrial septal defects are limited. The purpose of our retrospective study was to report the effectiveness of transcatheter closure in patients with various morphological types of atrial septal defect other than centrally located defects within the oval fossa using the CardioSEAL and CardioSEAL-Starflex occluder. A total of 91 patients (age 1.5-71 years, median 6 years) underwent transcatheter closure. On the transesophageal echocardiogram, defect size varied from 6 to 18 mm with an estimated stretched diameter of 11 to 24 mm, median 15 mm; the ratio of the stretched diameter to septal length ranged from 0.28 to 0.68. Mean follow-up was 28.7 +/- 11,9 months (range 3-46 months). Isolated secundum-type defects were present in 59 patients (65%), multiple septal defects including patients with perforated atrial septal aneurysms and defects with deficient atrial rim in 32 patients (35%). Occlusion rate using device diameters from 23 to 40 mm increased from 66% (60/91 patients) immediately after implantation to 86% (48/56 patients) 24 months after implantation. Patients with isolated secundum-type defects presented with a significantly higher primary closure rate (45/59 patients, 76%) compared to patients with various defect morphology. Closure rate did not depend on the type of implanted device modification. No thrombus formation, sustained atrial arrhythmia or infective endocarditis occurred. Serial transthoracic echocardiographic findings revealed protrusion of one left-sided arm onto the right atrial aspect in 5 patients; malposition of one right-sided superior arm of the device was observed in 7 patients. Fluoroscopy showed single fatigue fracture in 7 patients (7.7%) within the first 6 months after implantation. These results demonstrate that transcatheter closure with the double umbrella device was effective and safe on medium-term follow-up and could be extended to atrial septal defects of various morphology.

We sought to analyse the long-term follow-up after primary repair of tetralogy of Fallot in infancy in the first year of life, paying particular attention to growth of the pulmonary arteries and the need for reintervention. We performed a combined retro- and prospective echocardiographic study, including measurements of the pulmonary valve and right and left pulmonary arteries, indexed to the square root of body surface area, in 62 patients prior to primary repair, 18 to 24 months after this event, and at the most recent follow-up, with a mean of 80.4+/-24 months. Of these, 38 patients, with an age at operation of 5.0+/-3.4 months, had presented preoperatively with hypoxic spells or increasing cyanosis. The remaining 24 patients had been asymptomatic, with adequate flow of blood to the lungs. Their age at elective operation was 7.4+/-3.0 months. A transannular patch was needed in 37 patients (63%). There were 3 early postoperative deaths (4.8%). Cross-sectional echocardiography revealed a significantly smaller diameter for the pulmonary valve in patients who had been symptomatic preoperatively compared to the asymptomatic patients (1.09 versus 1.3 cm/BSA0.5; p = 0.019). The diameters of the right and left pulmonary arteries did not differ significantly between the groups. Examination of echocardiographic data obtained 18 to 24 months postoperatively in 43 patients revealed a significant increase in the diameter of the pulmonary arteries; 0.83+/-0.17 cm/BSA0.5 versus 1.1+/-0.26 cm/BSA0.5 for the diameter of the right pulmonary artery, 0.85+/-0.2 cm/BSA0.5 versus 1.0+/-0.25 cm/BSA0.5 for the left pulmonary artery. On comparison between individuals, 18.6% and 25.6% of the patients, respectively, did not show any change in the diameters of their right and left pulmonary arteries, whereas the increase in diameter reached or exceeded the measurements in normal controls in 55.8% and 46.5% of the patients, respectively. On recent follow-up, with a range from 56 to 147 months, no further increase in the indexed diameters of the pulmonaries could be documented in 21 of 33 patients. Of these, 9.1% and 18.2%, respectively, presented with small right and left pulmonary arteries compared with measurements obtained in normal controls. Moderate pulmonary incompetence was found on colour flow mapping in one-third. Of 56 longterm survivors, 8 (14%) had required reinterventions, which were surgical in 6 and achieved by transcatheter techniques in the other 2 patients. Thus, primary correction of tetralogy of Fallot in infancy, with restoration of normal pressures and flows, resulted in sustained increase in the diameters of the right and left pulmonary arteries. It allowed for early normal development of the proximal pulmonary arterial system in most patients regardless of their age and symptomatic status at operation. Patients with persistent subnormal diameter of the pulmonary arteries did not present with significantly elevated right ventricular pressure. Early one-stage repair of tetralogy of Fallot in infancy was associated with a low rate of reinterventions.

Background: The purpose of the present study was to determine the role of a novel, noncontact mapping system for assessing a variety of atrial reentrant tachycardias (ART) in patients after the surgical correction of congenital heart disease., Methods and Results: In 14 patients, an electrophysiological study using the Ensite 3000 system was performed to assess ARTs resistant to medical treatment. Sixteen different forms of ART were inducible in the 14 patients studied. The reentrant circuit of all ARTs could be characterized and localized with respect to anatomic landmarks such as atriotomy scars, intraatrial patches/baffles, and cardiac structures. In 15 of the 16 ARTs (in 13 of the 14 patients), a target area of the reentrant circuit for radiofrequency current application (ie, an area of conduction between 2 anatomical obstacles such as surgical barriers and cardiac structures of electrical isolation) could be localized within the systemic venous atrium. Nine patients exhibited macroreentry, and 4 showed microreentry. In 12 patients, ART could be terminated by creating linear radiofrequency current lesions (75 degrees C, 180 to 390 s). Completeness of linear lesions after radiofrequency current delivery was proven by analyzing color-coded isopotential maps of atrial activation while applying atrial pacing techniques. The mean duration of the procedures was 286 minutes (range, 130 to 435 minutes); fluoroscopy time ranged from 7 to 33.8 minutes (mean, 17.4 minutes)., Conclusions: In patients with ART after the surgical correction of congenital heart disease, the use of the noncontact mapping system allows for characterization of the tachycardia and guidance for effective radiofrequency current delivery.

Data on long-term follow-up for closure of so-called secundum type" atrial septal defects within the oval fossa using recently developed devices are limited, and results focused on presence of residual shunting. The purpose of our study was to report the experience from a single center establishing the effectiveness of transcatheter closure in patients with various types of defect other than those located centrally within the oval fossa. A total of 72 patients was included in this study. On transesophageal echocardiography, the size of the defects varied from 6 to 18 mm, with estimation of the stretched diameter from 11 to 21 mm. The ratio of stretched diameter to the extent of the residual septum ranged from 0.28 to 0.54. Mean follow-up was 30.5+7.4 months, with a range from 13 to 42 months. The rate of closure using devices with diameters from 28 to 40 mm increased from 80% immediately after implantation to 93% in the 57 patients examined 24 months after implantation. For further analysis, we compared the 44 patients with a solitary, centrally located, defect to 28 having morphological variations, including superiorly located defects with deficient superior and aortic rims, multifenestrated and aneurysmal defects, or isolated additional defects. There was no incidence of formation of thrombus, sustained atrial arrhythmia, or infective endocarditis. Residual shunting was not influenced by location or morphology of the defects, but increased with size, stretched diameter, and the ratio of pulmonary to systemic flows. Serial transthoracic echocardiographic findings revealed malposition of one right-sided superior arm of the device in 8 patients, while protrusion of one left-sided arm onto the right atrial aspect was observed in 3 patients. Fluoroscopy showed fatigue fracture of a single arm in 7 patients (9.7%) within the first 6 months after implantation. These results demonstrate that transcatheter closure with the non self-centering double umbrella device was effective and safe on medium-term follow-up, and could be extended to defects within the oval fossa having various morphologies. Residual shunting resolved with time, and was not related to either morphology or the position of the device.

A transcatheter technique is described for stabilization and retrieval of an embolized Amplatzer device, which was inadvertently deployed in the left atrium of a 2-year-old girl with hemodynamically significant atrial septal defect within the fossa ovalis. Since surgery was not available for immediate device retrieval, transcatheter means were chosen to prevent embolization of the device into the mitral valve. After stabilizing the device by creating a guidewire circuit through the wire mesh of the right atrial disk, the Amplatzer device was repositioned through the interatrial defect by snaring the microscrew. No residual shunting and perfect device position was recorded during Doppler echocardiography directly after the procedure as well as 1, 2, and 120 days after the intervention. Cathet. Cardiovasc. Intervent. 51:297-300, 2000., (Copyright 2000 Wiley-Liss, Inc.)

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.

Unlabelled: A coronary sinus defect results in a communication of variable size between the coronary sinus (CS) and the left atrium (LA). It is in most cases associated with a persistent left superior vena cava (l-SVC) and an atrial septal defect of the coronary sinus type and often part of a more complex cardiac malformation. If the CS to LA fenestration is the single cardiac defect, this anomaly provides the basis of an unusual form of interatrial communication, which may cause diagnostic difficulties. Two patients with this rare anomaly are reported. A 9-year-old boy presented with history of mild cyanosis pronounced after physical activity (SaO2 < 90%) since the age of 3. Comprehensive pulmonary and cardiological diagnostic procedures in the referring hospital yielded normal findings. Finally, a 1-SVC draining into a mildly dilated coronary sinus could be demonstrated echocardiographically. Contrast echocardiography revealed a right-to-left-shunt at atrial level. Diagnosis of a partially unroofed coronary sinus was subsequently confirmed during cardiac catheterization. The second patient was primarily diagnosed echocardiographically to have an atrial septal defect of the primum type (ASD I) in the first year of life. After pulmonary vascular markings on chest-x-ray had increased, she was assigned to our hospital for cardiac catheterization. Slight arterial desaturation (SaO2 88%) was present due to a large l-SVC anomalously draining into the LA, the innominate vein was missing. The roof of the coronary sinus was predominantly absent. Combined with a large atrial septal defect of the coronary sinus type, this resulted in significant left-to-right-shunt and right-to-left-shunt at atrial level due to a pure unroofed coronary sinus., Conclusion: A coronary sinus defect with partial or complete unroofing of the CS and a persistent l-SVC should be considered in the differential diagnosis of an otherwise unexplained systemic desaturation and in patients with both left-to-right-shunt and right-to-left-shunt at atrial level.

We report findings in spiral-CT from 11 adolescent or adult patients after atrial switch operation. The results demonstrate that computed tomography, particularly with the use of three-dimensional surface reconstruction, is very useful as a highly sensitive procedure for the detailed depiction of residua and sequelae after inflow correction for complete transposition. Especially systemic venous obstruction (SVO) is a well know complication following Mustard procedure for transposition of the great arteries. Demonstration of such obstruction is important, since the recognition and quantification of caval obstruction by clinical techniques is unreliable and indeed often impossible. Imaging procedures such as spiral computed tomography are important for this purpose. Advantages of spiral computed tomography, particularly with three-dimensional reconstruction, are discussed. The images were compared with findings in echocardiography and/or angiography affecting the site of operation.

Background: Operative resection is the treatment of choice for native aortic coarctation in most institutions. The ideal timing for elective repair is still a matter of debate. This study evaluated one institution's results with resection and end-to-end anastomosis in the first year of life., Methods: Between January 1987 and December 1993, 46 neonates and infants with functionally isolated aortic coarctation underwent operative resection and end-to-end anastomosis. For the patients included in the study, all hospital records, catheterization data, and operative protocols were evaluated for retrospective analysis. To obtain valid follow-up information, all patients were systematically seen on an outpatient basis during 1994., Results: After a mean follow-up of 49 +/- 24 months (range, 13 to 95 months), recoarctation (arm-leg blood pressure gradient > 20 mm Hg) occurred in 5 of 26 patients with neonatal operations (19%) and in 1 of 20 patients with operations in infancy (5%, p = not significant). Four of these 6 children with recoarctation needed reintervention. The other 2 patients had only mild recoarctation (gradients of 22 and 30 mm Hg, respectively) and were not treated. In all 6 patients, recoarctation was diagnosed within the first 6 months postoperatively. During the whole follow-up period, right arm systolic blood pressures slightly above the 90th percentile of normal developed in 11 of the patients (24%) (7 in the group with neonatal operation and 4 after operation in infancy; p = not significant)., Conclusions: Resection with end-to-end anastomosis was shown to be an adequate therapeutic strategy for isolated aortic coarctation in neonates and infants. The results indicate that already beyond the neonatal age, there is a relatively low incidence of recoarctation.

Introduction of the atrial baffle procedure (Mustard, Senning) more than 30 years ago has dramatically improved the prognosis of patients with transposition of the great arteries. However, a range of late complications can occur even in clinically asymptomatic patients including: superior and inferior limb obstruction, pulmonary venous obstruction, residual baffle-leakage, left ventricular outflow tract obstruction, tricuspid valve regurgitation, right ventricular dysfunction and postoperative dysrhythmia. As an increasing number of patients has reached adulthood an accurate noninvasive technique is needed for routine follow-up studies. In most patients transthoracic two-dimensional (2D) and Doppler echocardiography allows a complete investigation of postoperative hemodynamic abnormalities -- in particular assessment of atrial baffle function. In adult patients with restricted echo-windows transoesophageal echocardiography may provide a more detailed assessment of atrial baffle morphology and function; TEE seems to be superior compared to angiography and magnetic resonance imaging. Furthermore TEE is a reliable guiding and monitoring technique during interventional cardiac catheterization procedures such as balloon dilatation or stent implantation and allows further surveillance. Even if compared to other noninvasive diagnostic procedures, 2D and Doppler echocardiography (transthoracic/transoesophageal) are still the most important and accurate diagnostic methods for routine investigation of the postoperative anatomy and potential late complications in patients who underwent an atrial baffle procedure.

Background: As young age at modified Fontan operation was thought to be a preoperative risk factor for poorer survival, we studied early and intermediate outcome in our young patient group., Methods: Results in children less than 3 years of age (group I; n = 26; age range, 7 to 35 months) were compared with those in older patients (group II; n = 46; age range, 36 to 219 months). For both groups there was no significant difference with regard to preoperative pathology and hemodynamics., Results: With an overall mortality of 9.7% (7/72) there was no significant difference for both groups. Group I (n = 23) and group II (n = 42) survivors did not differ with respect to early postoperative incidence of atrial dysrhythmias, duration and volume of pleural effusion, or incidence of reoperation. Results on intermediate follow-up (group I, 31 +/- 14 months; group II, 44 +/- 20 months) demonstrated a relatively low incidence of late atrial dysrhythmias (12.3%; 8/65). Late atrioventricular valve insufficiency was significantly more frequent in group I patients., Conclusions: With similar preoperative anatomic and hemodynamic parameters including 68% of patients with complex univentricular heart, total cavopulmonary anastomosis could be performed in patients less than 3 years of age with good clinical and hemodynamic results, as achieved in older patients.

Pulmonary artery sling is often associated with tracheal stenosis. In many cases repair of the vascular anomaly alone does not relieve dyspnea. Primary one-stage repair with long segment tracheal resection (2.4 cm) and relocation of the left pulmonary artery using cardiopulmonary bypass and deep hypothermic circulatory arrest is described in a 6.5-month-old girl weighing 6.5 kg. This technique resulted in normal ventilation and pulmonary flow distribution.

Aortic thrombosis during neonatal period is rare and usually related to an umbilical artery catheter. In an infant (birth weight 3110 gm) with complete thrombosis of the abdominal aorta distal to the renal arteries of unknown etiology we started a fibrinolytic therapy with streptokinase at the age of 12 days (streptokinase intravenously with an initial loading dose of 3000 U/kg, maintenance 1000 U/kg/hr for 2 days). This was followed by a heparinisation (10,000 U/m2/d, 24 hours later 200 U/kg/d). 72 hours after onset of therapy there was no blood pressure difference between upper and lower limbs. Side-effects of therapy did not occur; the success of medical treatment continued after termination of the heparinisation.

In order to evaluate postoperative sequelae and ventricular function after Mustard-operation in patients with transposition of the great arteries (TGA), 30 patients were assessed by magnetic resonance imaging in EKG-triggered spin-echo (SE) and gradient-echo (GE) technique. Twenty-three patients, aged 4.7 to 15.8 years, had transposition of the great arteries with intact ventricular septum with or without left-ventricular outflow tract obstruction (TGA+IVS +/- SPS). Seven patients aged 9.5 to 21.7 years had transposition of the great arteries with ventricular septal defect (TGA+VSD). Five patients showed a residual baffle leak, one had a pulmonary venous obstruction, five an obstruction at the caval veins, 13 a left-ventricular outflow tract obstruction, and 14 a tricuspid regurgitation. Right-ventricular enddiastolic volume in patients with TGA+VSD (77.0 +/- 25.5 ml/m2) was significantly higher than in patients with TGA+IVS +/- SPS (61.2 +/- 12.0 ml/m2). In TGA+VSD right-ventricular ejection fraction (47.6 +/- 13.0%) was significantly lower than in patients with TGA+IVS +/- SPS (56.7 +/- 10.7%). The ratio of muscle masses of right to left ventricle was 1.8:1 in patients with TGA+IVS +/- SPS and 2.5:1 in patients with TGA+VSD. In conclusion, after Mustard-operation in patients with transposition of the great arteries (TGA) magnetic resonance imaging provides a comprehensive and noninvasive assessment of postoperative sequelae, residuae, and ventricular function and will, therefore, become the method of choice for postoperative evaluation.

With the use of dedicated pediatric single-plane probes, transesophageal echocardiography was performed prospectively in 102 children to determine the potential value of this new technique in the diagnosis (40 patients) and the follow-up of congenital heart disease (29 patients) and as a monitoring technique during the perioperative period or interventional cardiac catheterization (33 patients). The findings were correlated with precordial studies (102 patients), cardiac catheterization (82 patients), epicardial ultrasound findings (18 patients), or surgical inspection (34 patients). The age at investigation ranged from 2.5 months to 14.9 years (mean age, 5.2 years); weight ranged from 3.7 to 52 kg (mean weight, 19.1 kg). Additional information was obtained in 49 patients (48.4%) and was relevant for patient management in 21 patients (20.6%). Major areas of improved diagnostic insight included the following: (1) systemic and pulmonary venous return, (2) atrial morphology, (3) atrioventricular junction abnormalities, (4) left ventricular outflow tract disease, (5) atrial baffle function, and (6) the Fontan circulation. Inherent limitations were as follows: (1) the semiinvasive character of the procedure, (2) the need for heavy sedation or general anesthesia, and (3) the limited imaging planes provided. Transesophageal echocardiography in children can provide important additional diagnostic information on a wide spectrum of congenital heart lesions. The technique would appear to be a highly valuable adjunct to the diagnostic armentarium of the pediatric cardiologist.

Information obtained from transthoracic and transesophageal echocardiography (two-dimensional echocardiography with spectral Doppler and color flow imaging) was compared in 17 patients with major congenital abnormalities of the atrioventricular (AV) junction (10 discordant AV connections, 1 criss-cross connection, 5 absent right connections and 1 absent left connection). The findings by either technique were correlated with findings at cardiac catheterization (12 patients) and at surgery (5 patients). In two of six patients with an absent AV connection as defined by transthoracic echocardiography, transesophageal imaging demonstrated an imperforate AV valve. In 11 of 11 patients with a discordant or criss-cross connection, assessment of AV valve and ventricular morphology (by defining the chordal attachments of both AV valves) was possible with transesophageal echocardiography (3 of 11 patients by transthoracic echocardiography); chordal straddling was detected in 1 patient and excluded in 3 others with an associated inlet ventricular septal defect. Anomalous pulmonary venous connection (one patient), atrial septal defect (three patients) and subpulmonary stenosis (five patients) were better assessed by transesophageal imaging, and atrial appendage morphology could be demonstrated in all. The transesophageal technique was less useful in demonstrating the anterior subaortic infundibulum or aortopulmonary shunt (two patients). Although systemic ventricular function could be assessed by either method with use of short-axis M-mode scans, transesophageal pulsed Doppler interrogation of AV valve and pulmonary venous flow patterns provided clues to diastolic dysfunction of the systemic ventricle.

In 94 consecutive patients undergoing surgical repair of congenital heart defects the results of intraoperative (after cardiopulmonary bypass) epicardial two-dimensional and Doppler color flow imaging were compared with those of sequential transthoracic echocardiography performed within 24 h of surgery and again before hospital discharge to define the precise role of intraoperative imaging. In 6 of 7 patients with a residual defect requiring immediate surgical revision, intraoperative imaging correctly identified the defect; spectral Doppler imaging underestimated or did not identify a residual outflow tract gradient in 17 patients. Left atrioventricular (AV) valve regurgitation after repair of complete AV septal defect was underestimated in three patients. Although intraoperative documentation of good ventricular function was usually associated with a good outcome, in three patients poor systemic ventricular function after cardiopulmonary bypass was not associated with early mortality. A minor degree of shunting around the patch was a common finding on epicardial and early postoperative imaging and persisted at the time of hospital discharge in 17 of 46 patients who had undergone patch closure of a ventricular septal defect as part of the surgical procedure. Additional trabecular septal defects were missed on color flow imaging after cardiopulmonary bypass in three patients, one of whom required subsequent reoperation. Although intraoperative two-dimensional and color flow imaging permitted the recognition of the majority of residual defects requiring immediate revision, residual outflow obstruction or AV valve regurgitation was usually underestimated.

Twenty-eight patients (age range, 0.7 to 65 years; median age, 6.1 years) who were undergoing correction for congenital heart disease were entered into a prospective study with both intraoperative transesophageal and epicardial ultrasound to determine the relative values of these techniques before and after bypass surgery. Introduction of the transesophageal probe was successful in 26 patients (93%); children were studied with use of dedicated pediatric transducers. Epicardial studies were performed in all 28 patients. Epicardial studies allowed for higher resolution imaging and a more complete assessment before bypass surgery of the intracardiac morphological condition (ventricular septum and right ventricular outflow tract) than the assessment that was obtained by the transesophageal approach. In the period immediately after bypass surgery, the transesophageal technique allowed a more detailed insight into atrioventricular valve function (valvar regurgitation [five patients] and ventricular inflow patterns) and the continuous monitoring of left ventricular function and volume. Residual interventricular shunting (three patients) or residual outflow tract obstruction (four patients) could not be reliably documented by transesophageal studies. It is concluded that intraoperative transesophageal and epicardial ultrasound in surgery for congenital heart disease are complementary rather than alternative techniques.

Following a Mustard's procedure, transoesophageal echocardiography allowed the visualization of all 4 pulmonary veins in 7/12 patients (mean age 14.8 years) and of three veins in a further 2 patients. Both upper pulmonary veins could be visualized consistently. No patient had an isolated pulmonary venous stenosis. In 9 patients in sinus rhythm, computer analysis of Doppler tracings from the left upper pulmonary vein showed significantly lower systolic peak velocities (mean 0.39 +/- 0.10 m/s) and time velocity integrals (mean 6.9 +/- 1.66 cm) than in normal subjects (mean 0.6 +/- 0.09 m/s and 14.4 +/- 2.97 cm respectively; P less than 0.001). We postulate that this is due to compromised atrial relaxation and compliance. In contrast, patients in junctional rhythm (mean 10.7 vs. 7.7 cm in normal subjects). Flow reversal during early ventricular systole (due to tricuspid regurgitation or atrial contraction after retrograde conduction during junctional rhythm) was detected in 6/12 patients. These results confirm that the transoesophageal approach should allow the identification of isolated pulmonary venous obstruction after a Mustard procedure. In addition. detailed analysis of tracings of flow in the pulmonary veins can document the presence of compromised atrial relaxation and help to evaluate the severity of tricuspid regurgitation. It may provide a new index with which to assess impaired systemic ventricular function.

Previous methods used to assess atrial baffle function after correction of transposition of the great arteries have included precordial echocardiography and cardiac catheterization. To evaluate whether single plane transesophageal echocardiography might provide additional information, its findings were correlated with information derived from both precordial echocardiography and cardiac catheterization in 15 patients (14 Mustard procedures, 1 Senning procedure) aged 4.2 to 33 years (mean 16.3). Precordial ultrasound with combined imaging, color flow mapping and pulsed Doppler ultrasound visualized the supramitral portion of the common systemic venous atrium in every case but could identify only superior limb obstruction in three of six patients, mid-baffle obstruction in zero of two and inferior limb obstruction in zero of two patients. Transesophageal studies with use of the same range of ultrasound methods demonstrated superior limb obstruction (severe in four, mild in two) in six of six patients, mid-baffle obstruction in two of two and inferior limb obstruction in two of two patients. The entire pulmonary venous atrium was equally well interrogated by either ultrasound approach, with both identifying three cases (two mild, one moderate) of mid-pulmonary venous atrium obstruction. However, individual pulmonary vein velocity profiles could only be recorded by transesophageal pulsed Doppler ultrasound. Precordial studies identified baffle leaks (1 large, 2 small) in only three patients, whereas transesophageal studies identified 11 such baffle leaks (1 large, 10 small), which were multiple in two patients. It is concluded that transesophageal echocardiography provides a more detailed and accurate assessment of atrial baffle morphology and function than is provided by either precordial ultrasound or cardiac catheterization.(ABSTRACT TRUNCATED AT 250 WORDS)

From 1975-1986 6 boys with congenital nephrogenic diabetes insipidus were diagnosed at the age of 3 months to 10 years. Symptoms appeared within the first few weeks of life. The diagnosis was confirmed by polyuria, low urinary osmolality (97-225 mosm/kg H20), hypernatraemia (max. 171 mmol/l) and the missing response to vasopressin. The treatment was variable; 4 boys received only hydrochlorothiazide (2-2.5 mg/kg/d) which lead to a reduction of the daily urinary volume of 26-44%. Hyperelectrolytaemia disappeared and a normal thriving could be achieved. Later an additional treatment with indomethacin (2 mg/kg/d) was necessary in 3 boys because of an increase of polyuria; there was a further reduction of the daily urinary volume of 50-60%. The combination of hydrochlorothiazide and indomethacin in the treatment of the congenital nephrogenic diabetes insipidus was well tolerated and seems to be--especially during the first few years of life--a necessary and effective treatment which allows a normal thriving and psychointellectual development.