[Cardiac MRI in the follow-up of adult congenital cardiomyopathy patients]. / IRM cardiaque dans le suivi des cardiopathies congénitales à l'âge adulte.

Magnetic resonance imaging is a rapidly developing modality in cardiology. It offers an excellent image definition and a large field of view, allowing a more accurate morphological assessment of cardiac malformations. Due to its unique versatility and its ability to provide myocardial tissue characterization, cardiac magnetic resonance (CMR) is now recognized as a central imaging modality for a wide range of congenital heart diseases, including assessment of post-surgical cardiac anatomy, quantification of valvular disease and detection of myocardial ischemia. CMR provides useful diagnostic information without any radiation exposure, and improves the global management of patients with congenital heart disease.

Preoperative acidosis and infant development following surgery for congenital heart disease.

OBJECTIVE: Prenatal diagnosis has been shown to decrease pre-operative acidosis and might prevent the occurrence of disturbed developmental outcome. The aim of this study is to evaluate parameters for acidosis and their predictive value on developmental outcome in newborns with congenital heart disease. METHODS: A total of 117 patients requiring surgery for structural heart disease in the first 31 days of life were included. Diagnosis was established either pre- or postnatally. Preoperative values of lactate, pH and base excess levels were compared to the occurrence of disturbed developmental outcome, i.e. an underperformance of more than 10% on the P90 of a standardized Dutch developmental scale. Patients were divided into groups according to blood levels of acidosis parameters, using receiver operating characteristics curves to determine cut-off values for pH, base excess and lactate. RESULTS: No significant difference in developmental outcome was found using values for pH or base excess as a cut-off level. Preoperative lactate values exceeding 6.1 mmol/l resulted in a significant increase in impaired development compared to infants with a pre-operative lactate lower than 6.1 mmol/l: 40.9% vs 15.1% in (p=0.03). CONCLUSIONS: Pre-operative lactate values might have a prognostic value on developmental outcome in newborns with congenital heart disease. The limited prognostic value of pH can be explained by the fact that pH can be easily corrected, while lactate better reflects the total oxygen debt experienced by these patients.

[Transposition of the great arteries: long-term follow-up]. / Transposition des gros vaisseaux: suivi a long-terme.

The management of transposition of the great arteries has changed importantly over the last decades. New techniques are employed for the diagnosis and surgical intervention has improved. This has lead to an increasing number of long-term survivors, who require specialised and focussed follow-up, depending on their type of surgical repair Mustard/Senning of arterial Switch. The long-term problems vary for each type of repair and require a specific approach, pharmacotherapy, catheter intervention or surgery. These complex sequellae implicate that transposition patients are never completely cured and probably will have a limited life span.

Sotalol in the treatment of fetal dysrhythmias.

BACKGROUND: Fetal tachycardia may cause hydrops fetalis and lead to fetal death. No unanimity of opinion exists regarding the optimum treatment. This study evaluates our experience with transplacental sotalol therapy to treat fetal tachycardias in terms of safety and efficacy. METHODS AND RESULTS: The charts of 21 patients who were treated with sotalol for fetal tachycardia were reviewed. Ten fetuses had atrial flutter (AF), 10 had supraventricular tachycardia (SVT), and 1 had VT. Hydrops fetalis was present in 9 fetuses. Drug treatment was successful in establishing sinus rhythm in 8 of 10 fetuses with AF and in 6 of 10 fetuses with SVT. The mortality rate in this study was 19% (4 of 21 fetuses; 3 had SVT and 1 had AF); 3 deaths occurred just days after the initiation of sotalol therapy, and 1 occurred after a dosage increase. At birth, tachycardia was present in 6 infants. Two patients who converted to sinus rhythm in utero suffered from neurologic pathology postnatally. CONCLUSIONS: Fetal tachycardia is a serious condition in which treatment should be initiated, especially in the presence of hydrops fetalis. The high success rate in fetuses with AF suggests that sotalol should be considered a drug of first choice to treat fetal AF. The low conversion rate and the fact that 3 of the 4 deaths in this study occurred in fetuses with SVT indicate that the risks of sotalol therapy outweigh the benefits in this group and that sotalol should, therefore, be limited in the treatment of fetal SVT.

Management outcome and follow-up of fetal tachycardia.

OBJECTIVES: The aim of this study was to evaluate fetal tachycardia and the efficacy of maternally administered antiarrhythmic agents and the effect of this therapy on delivery and postpartum management. BACKGROUND: Sustained fetal tachycardia is a potentially life-threatening condition in which pharmacologic therapy is reported to be effective. There is ongoing discussion about optimal management. METHODS: A group of 51 patients with M-mode echocardiographically documented fetal tachycardia was studied retrospectively. RESULTS: Thirty-three fetuses had supraventricular tachycardia; 15 had atrial flutter; 1 had two episodes of both; and 2 had ventricular tachycardia. Fetal hydrops was seen in 22 patients. Thirty-four fetuses received maternal therapy with either digoxin or flecainide as the first administered drug (additional drugs were given in 12). Drug treatment was successful in establishing acceptable rhythm control in 82% (84% without, 80% with hydrops). In the latter group the median number of drugs and number of days to conversion were higher. Three patients with fetal hydrops died. In 50% of cases, tachycardia reappeared at delivery: 9 neonates presented with atrial flutter, 14 with supraventricular tachycardia and 1 with ventricular tachycardia. Seventy-eight percent of the group had pharmacologic therapy by 1 month of age and 14% by 3 years. CONCLUSIONS: Fetal tachycardia can be treated adequately in the majority of patients, even in the presence of hydrops, and therefore emergency delivery might not be indicated. Digoxin and flecainide were drugs of first choice and produced no serious adverse effects in this series of patients. The majority of patients do not require prolonged therapy.

Atrial flutter in the perinatal age group: diagnosis, management and outcome.

OBJECTIVES: The aim of this retrospective study was to evaluate perinatal atrial flutter (AF) and the efficacy of maternally administered antiarrhythmic agents, postpartum management and outcome. BACKGROUND: Perinatal AF is a potentially lethal arrhythmia, and management of this disorder is difficult and controversial. METHODS: Forty-five patients with documented AF were studied retrospectively. RESULTS: Atrial flutter was diagnosed prenatally in 44 fetuses and immediately postnatally in 1 neonate. Fetal hydrops was seen in 20 patients; 17 received maternal therapy, 2 were delivered and 1 was not treated because it had a severe nontreatable cardiac malformation. In the nonhydropic group of 24 patients, 18 were treated and the remaining 6 were delivered immediately. In the hydropic group, 10 received single-drug therapy (digoxin or sotalol) and 7 received multidrug therapy. In the nonhydropic group, 13 received a single drug (digoxin or sotalol) and 5 received multiple drugs. One patient with rapid 1:1 atrioventricular conduction (heart rate 480 beats/min) died in utero and another died due to a combination of severe hydrops because of the AF, sotalol medication, stenosis of the venous duct and hypoplastic placenta. Of the 43 live-born infants, 12 were in AF at birth. Electrical cardioversion was successful in eight of nine patients. No recurrences in AF have occurred beyond the neonatal period. Four patients with fetal flutter and hydrops showed significant neurological pathology immediately after birth. CONCLUSIONS: Fetal AF is a serious and threatening rhythm disorder, particularly when it causes hydrops, it may be associated with fetal death or neurological damage. Treatment is required and primarily aimed at reaching an adequate ventricular rate and preferably conversion to sinus rhythm. Digoxin failed in prevention of recurrence at time of delivery in a quarter of our patients, whereas with sotalol no recurrence of AF has been reported, suggesting that class III agents may be the future therapy. Once fetuses with AF survive without neurological pathology, their future is good and prophylaxis beyond the neonatal period is unnecessary.

Dilated cardiomyopathy in isolated congenital complete atrioventricular block: early and long-term risk in children.

OBJECTIVES: We sought to identify the risk factors predicting the development of dilated cardiomyopathy (DCM) in patients with isolated congenital complete atrioventricular block (CCAVB). BACKGROUND: Recently evidence has emerged that a subset of patients with CCAVB develop DCM. METHODS: This was a retrospective study of 149 patients with CCAVB who had heart size and left ventricular (LV) function assessed by echocardiography and chest radiography over a follow-up period of 10 +/- 7 years. RESULTS: Nine patients developed DCM at the age of 6.5 +/- 5 years. No definite cause could be identified. In these nine patients, CCAVB was diagnosed in eight at 23 +/- 2.3 weeks gestation and in one at birth. Maternal SSA/SSB antibodies were confirmed in seven of the nine patients. Pacemakers were implanted in eight patients in the first month and in one patient at five years of age. The initial left ventricular end-diastolic dimension (LVEDD) was in the 96th +/- 2.6 percentile and the cardiothoracic (CT) ratio was 64 +/- 3.8% in the nine patients who developed DCM, and differed significantly in patients with CCAVB (p < 0.005) who did not develop DCM. The LVEDD and CT ratio did not decrease in the patients with CCAVB and DCM, but decreased significantly in the patients with CCAVB without DCM (p < 0.001) once pacing was initiated. Two patients with DCM died within two months of diagnosis; one patient is neurologically compromised; two patients received a heart transplant; and four patients are listed for heart transplantation. CONCLUSIONS: Isolated CCAVB is associated with a long-term risk for the development of DCM. Risk factors may be SSA/SSB antibodies, increased heart size at initial evaluation and the absence of pacemaker-associated improvement.

[Treatment of congential heart disease with interventional catheterization]. / Traitement des cardiopathies congénitates par cathétérisme interventionnel.

The development in pediatric interventional cardiology has changed the therapeutic strategy for many patients with congenital heart disease. Interventional catheterization became the first choice for valvuloplasty, angioplasty and collateral vessel occlusion. Effective and safe transcatheter interventions exist for closure of atrial or ventricular septal defects and for patent ductus arteriosus. The progress in the technology used in the catheterization laboratory will permit to continue the expansion of the range of interventions performed without surgery. Pediatric cardiologists and congenital heart surgeons must understand each other's interventional techniques and how they can be used in a coordinated fashion. This interaction is essential for the optimal management of patients with both simple and complex congenital heart disease.

[Pediatrics. Advantages of prenatal diagnosis in congenital cardiopathies]. / Pédiatrie. Avantages du diagnostic prénatal dans les cardiopathies congénitales.

The diagnosis of a congenital heart disease can be made by fetal ultrasound as soon as the 12th week of pregnancy. The main indications for fetal echocardiography are maternal and fetal. Sequential approach is necessary to precise the anatomy and to elaborate a therapeutic strategy for that patient. For severe heart lesions delivery and postnatal care can be programmed, avoiding thus severe hypoxia and acidosis after birth. The prognosis of these children are thus much improved.

Fetal cardiac output estimated by Doppler echocardiography during mid- and late gestation.

Fetal cardiac output was quantitated by 2-dimensional (2-D) Doppler echocardiography. Circulatory dynamics in the fetus differ from circulatory dynamics after birth because right and left ventricles work in parallel. Therefore, volume flow was estimated over the mitral and tricuspid orifice separately. Serial 2-D echo Doppler studies were performed in 28 normal fetuses at 4-week intervals from 15 to 18 weeks of gestation to the time of parturition. Measurement of blood flow velocities through mitral and tricuspid valve orifices and measurement of the diameter of these valve orifices were obtained from apical 4-chamber views of the fetal heart. Angle-corrected mean temporal blood flow velocities increased linearly with gestational age, whereas the area of blood flow and calculated right and left ventricular output increased exponentially. The right ventricular blood flow velocities, areas of tricuspid orifice and calculated right ventricular outputs were significantly higher than those for the left ventricle when analyzed by paired t test (0.005 greater than 2p greater than 0.001). The ratio of right to left ventricular output decreased from 1.34 +/- 0.28 (+/- standard deviation) at 15 weeks to 1.08 +/- 0.28 at 40 weeks. The combined ventricular output of the fetus near term is approximately 1,735 ml/min, whereas flow indicated for estimated fetal weight is constant at a mean value of 553 +/- 153 ml/min/kg. Thus, 2-D echo Doppler provided a means of estimating fetal cardiac blood flow at the atrioventricular orifices, and blood flow measured at these orifices increased exponentially during gestation, with a larger output from the right ventricle than from the left ventricle.

Limits of reproducibility of blood flow measurements by Doppler echocardiography.

This study evaluates the variability of a quantitative Doppler echocardiographic method for blood flow calculation at the mitral and tricuspid orifice. Four subjects underwent 2-dimensional Doppler echocardiography during normal respiration and nonrespiration. Doppler recordings were integrated to determine mean temporal velocity (MTV) for each cardiac cycle separately. MTV during inspiration and expiration were compared, as were MTVs of 20 consecutive cycles during nonrespiration. Diameters of mitral and tricuspid orifice and interception angles were measured in 10 consecutive cycles at 4 predetermined moments. All results were averaged to a mean subject situation. MTVs were significantly (p less than 0.001) higher during expiration than during inspiration (12.4 and 11.0 cm-2) for the mitral orifice and lower (9.2 and 11.0 cm-2) for the tricuspid orifice. MTV at both orifices showed a significantly smaller variability (7.7% and 9.0%) during nonrespiration than during respiration (14.5% and 13.2%). Diameters of mitral orifice and tricuspid orifice were significantly (p less than 0.001) larger during diastole than during systole whereas standard error of the mean for both was 5.0%. Interception angles measured at mitral orifice are all close to 0 degrees and show minimal variability, while at the tricuspid orifice the angle varied from 15 degrees in diastole to 25.5 degrees in systole, constituting a significant difference in cosine (0.96 to 0.90).

Left atrioventricular valve after surgical repair in atrioventricular septal defect with separate valve orifices ("ostium primum atrial septal defect"): an echo-Doppler study.

Left atrioventricular (AV) valve dysfunction is the most frequent major postoperative hemodynamic complication in patients with AV septal defect. The anatomy and function of the left AV valve were investigated in 64 patients with separate valve orifices (ostium primum atrial septal defect) who had survived corrective surgery. M-mode and cross-sectional echocardiograms of the left AV valve were obtained. Doppler flow tracings were obtained at the left AV valve orifice to determine if regurgitation was present. The findings were related to the position of the commissures between the leaflets, the size of the 3 leaflets and the position of the papillary muscles. Left AV valve regurgitation was present in 29 of 51 patients (57%). These patients had a significantly different left AV valve leaflet configuration, characterized by a large mural leaflet and a small inferior bridging leaflet. The size of the superior bridging leaflet is not a determinant factor. Thus, the configuration of the left AV valve in AV septal defect is related to the postoperative functional result. Awareness of the echocardiographic anatomy may influence the surgical approach to this defect.

Anatomic and functional "obstruction" of the outflow tract in atrioventricular septal defects with separate valve orifices ("ostium primum atrial septal defect"): an echocardiographic study.

Left ventricular (LV) outflow tract (OT) obstruction can be treacherous in any form of atrioventricular (AV) septal defect. The properties of the LVOT were investigated echocardiographically in 64 patients with separate valve orifices ("ostium primum atrial septal defect") who had survived corrective surgery. M-mode and cross-sectional echocardiographic (echo) images were made of the LVOT. The degree of malalignment of the aorta with the ventricular septum, the left atrium-aortic ratio, the fractional LV shortening and the diameter of the LVOT were recorded. Fixed anatomical obstruction was found in 3 patients, consisting of muscular bands or abnormal attachment of tension apparatus. Malalignment of the aorta with the ventricular septum was found in 62% of the patients. The diameter of the LVOT was smaller than that of the aortic root in 71% of the cases. The mean diameter of the LVOT was 92 +/- 27% (range 35 to 143%) of the aortic root diameter. Because its walls are mainly muscular, the LVOT constricts during systole. The mean end-systolic diameter of the LVOT was 77 +/- 22% (range 23 to 129%) of the aortic root diameter. Sequential measurements showed that the LVOT constricted gradually, but the velocity of constriction in patients with the most severe narrowing showed a distinct maximum in the first fifth of systole. In conclusion, a series of elements contribute to a potentially perilous arrangement of the LVOT in patients with AV septal defect. This intrinsically narrow tunnel was constricted during systole by its muscular walls.(ABSTRACT TRUNCATED AT 250 WORDS)

Twenty-five years' experience with the arterial switch operation.

OBJECTIVE: The arterial switch operation was introduced in 1976 to correct transposition of the great arteries and has since replaced atrial palliation. This study examines the long-term outcomes of the arterial switch operation. METHODS: Included in the study were 195 patients who underwent the arterial switch operation from 1977 through June 2000. Patients were evaluated for functional class, pulmonary stenosis, left ventricular function, arrhythmias, aortic sequelae, and coronary disease. RESULTS: Overall perioperative mortality was 15%, but rates dropped in the last 5 years to 4% for complex transposition and 0% for simple transposition. Of 151 survivors, 2 died late; 1 died of persisting pulmonary hypertension and 1 died of ventricular fibrillation after coronary pathologic changes. At last follow-up, a total of 145 patients were in New York Heart Association functional class I and 4 patients were in class II. The most frequent complication was pulmonary stenosis, necessitating 45 reinterventions in 26 patients. Left ventricular dysfunction was noted in 5 patients. Arrhythmias were seen in 5 patients: 2 patients had ventricular fibrillation (1 died and 1 required a defibrillator implantation), 1 had sick sinus syndrome, 1 had atrial flutter, and 1 had a single attack of supraventricular tachycardia. Aortic valve incompetence was absent or trivial in 146 patients, 3 had mild incompetence, 1 had moderate incompetence, and 1 had severe incompetence. Coronary sequelae were found in 5 of the 61 patients who underwent angiography. CONCLUSION: Long-term clinical outcome of the arterial switch operation is good, and perioperative mortality is now low. Morbidity is dominated by pulmonary stenosis and coronary artery disease, with the potential for lethal arrhythmias.

Prenatal diagnosis of congenital heart disease affects preoperative acidosis in the newborn patient.

OBJECTIVES: Congenital heart disease is the leading cause of death in the first year after birth. Prenatal diagnosis of the disease can optimize the preoperative condition of the patient and may help in the prevention of acidosis. In this retrospective study we compared the occurrence of metabolic acidosis in patients with and without prenatal diagnosis of a congenital heart disease. METHODS: Data of 408 patients who needed an operation for congenital heart disease within 31 days of life were analyzed retrospectively. Arterial blood gases at fixed time intervals and worst blood gas of 81 patients with and 327 patients without a prenatal diagnosis were compared, categorizing the patients on ductus dependency, anticipated univentricular or biventricular repair, and left-sided, right-sided, or no heart obstruction. RESULTS: In the overall group significant differences in lowest pH, lowest base excess, and highest lactate level were found, with metabolic acidosis more common among the patients with a postnatal diagnosis. In the group of patients with ductus-dependent congenital heart disease, the difference between patients receiving a prenatal and those receiving a postnatal diagnosis was more significant than in the group with non-ductus-dependent lesions. Analyzing patients with right-sided, left-sided, and no obstruction separately, significant differences were found in the group with left-sided heart obstruction for lowest pH and base excess and in the group with right-sided heart obstruction for lowest base excess. CONCLUSIONS: Prenatal diagnosis of congenital heart disease minimizes metabolic acidosis in patients with congenital heart disease and may be associated with improved long-term outcome and prevention of cerebral damage among this fragile group of patients, although no significant effect on direct surgical outcome was encountered.

The surgical anatomy of the left ventricular outflow tract in atrioventricular septal defect.

The left ventricular (LV) outflow tract (OT) in atrioventricular (AV) septal defect is an important structure that paradoxically is hardly ever seen by a surgeon. The LVOT is prone to develop obstruction following surgical procedures, such as left AV valve replacement, that seemingly do not affect the LVOT itself. We examined 15 hearts with AV septal defects and noted the anatomical boundaries of the LVOT. Additionally, the LVOT was examined microscopically, and it was sectioned to replicate echocardiographic images. A sham operation was performed to show the extent of the proposed resection for AV valve replacement. The mean length of this area was 91.8 +/- 35.5% (range, 28.6 to 167.0%) of the diameter of the ascending aorta in our specimens of the Rastelli A variety. The mean diameter of the LVOT was 68.2 +/- 13.5% (range, 42.9 to 100.0%) of the diameter of the ascending aorta. The posterior wall of the OT can either be resected or widened. Resection seems to be opportune at AV valve replacement, whereas widening could be performed when the OT is intrinsically stenotic. When one fully appreciates the concept of a five-leaflet common valve, it is clear that the length of the OT depends on the extent of adherence between the superior bridging leaflet and the septal crest. In hearts that have two separate AV valve orifices, the OT is fully developed; there is no potential for interventricular shunting ("ostium primum defect"), because the superior bridging leaflet is always tightly adherent to the septal crest. AV valve replacement in these cases is especially hazardous.(ABSTRACT TRUNCATED AT 250 WORDS)

Management of ventricular septal defect with associated aortic incompetence.

Twenty-two patients with a ventricular septal defect and aortic incompetence underwent surgical repair. Mean age was 9.6 years (range, 9 to 15 years). Mean follow-up was 32.4 +/- 15.8 months. The ventricular septal defect was perimembranous in 19 patients and doubly committed or juxtaarterial in 3. Most showed only a small left-to-right shunt. Mean diameter was 11.9 +/- 4.8 mm. Aortic incompetence was mild in 6 patients, moderate in 4, and severe in 12. In 16 patients closure of the ventricular septal defect and aortic valvoplasty were both performed through the aortic root. There were no deaths. All patients retained normal sinus rhythm. No complete heart block was found. In 3 patients secondary aortic valve replacement was required for severe incompetence; in 1 the cause was bacterial endocarditis, in another technical failure, and in a third progressive incompetence over an 8-month period. All patients showed substantial clinical improvement, marked decrease or disappearance of the valvar incompetence, diminution of the left ventricular end-diastolic diameter, and decreased cardiothoracic ratio. We conclude that primary repair is the operation of choice for this combination of lesions. This can avoid or delay considerably valvar replacement. The left-sided approach proves safe and is our preferred technique.

A comparison of Blalock-Taussig, Waterston, and polytetrafluoroethylene shunts in children less than two weeks of age.

Results obtained with Blalock-Taussig, Waterston, and polytetrafluoroethylene (PTFE) shunts were compared in 67 cyanotic infants less than 2 weeks of age. A different shunt was preferably used at each of three institutions. The incidences of early shunt failure (3 out of 21, 14%), mortality after revision of early shunt failure (0 out of 3), and overall hospital mortality (1 out of 21, 5%) were all lowest for the PTFE shunt. Incidence of congestive heart failure secondary to excessive flow was comparable for the Blalock-Taussig and PTFE shunts, both of which were lower than the Waterston shunt. Cumulative probabilities of late shunt adequacy were calculated for hospital survivors. At 1 year, all shunts provided comparable adequate palliation (greater than 80%). Probability of late shunt failure was significantly higher (p = 0.04) for the PTFE shunt at 3.5 years. Results suggest that the PTFE shunt may be the safest and most effective shunt in neonates, but that elective shunt replacement or total repair may be warranted in the first or second year of life.

Usefulness of MRI for the pre-operative evaluation of the pulmonary arteries in Tetralogy of Fallot.

Adequate pre-operative evaluation of patients with Tetralogy of Fallot (TF) includes cine-angiography to delineate the pulmonary vasculature and the coronary artery anatomy and to demonstrate the presence of multiple ventricular septal defects (VSDs). All other information is obtained from color-Doppler-echocardiography. Magnetic resonance imaging (MRI), using the spin-echo sequence and cine-angiography was employed on 18 patients with TF, four of whom had aorto-pulmonary shunts. Mean age at MRI was 12.9 m (SD 2.3 m) and 14.3 m (SD 2.8 m) at cine-angiography. To compare MRI and cine-angiography we measured the ascending aorta, the main, the left and right pulmonary arteries and each structure at three levels. Diagnostic agreement between the two imaging methods was found if, for each modality, one of the three measurements in one structure differed by more than 40% from the other two measured in case of a local stenosis, and the diameter of the main pulmonary artery was less than 60% of the aorta to diagnose hypoplasia of the main pulmonary artery. There was close agreement between cine-angiography and MRI. With regard to the intracardiac anatomy, MRI was superior to color-Doppler-echocardiography in the depiction of aortic override and of right ventricular hypertrophy. In three cases local stenoses in the pulmonary arteries were detected by MRI and cine-angiography. Hypoplasia of the main pulmonary artery was detected by MRI in six patients and by cine-angiography in five patients. Cine-angiography missed one case of hypoplasia. In the remaining 11 patients normal findings were found by MRI and cine-angiography. For the demonstration of shunts, gradient-recalled-echo MRI is expected to give better results than the spin-echo sequence which depicted two out of four shunts in this series. Cine-angiography can be substituted by MRI in delineating the pulmonary arteries. New developments in MRI indicate the feasibility of delineating the coronary arteries.

MRI appearance of a double inlet and double outlet right ventricle with supero-inferior ventricular relationship.

This patient was diagnosed with a double inlet and double outlet RV with supero-inferior ventricular relationship, ventricular inversion was diagnosed on the basis of left-handed topology of the RV, and the straddling of the right-sided mitral valve over an anterior VSD, with its tension apparatus extending into the outflow tract of the RV. MRI was found to be superior to color Doppler echocardiography and contrast ventriculography in the segmental analysis leading to a full understanding of this complex case.