We undertook our study in order to evaluate right ventricular function and perfusion by conventional and contrast echocardiography in adults with congenitally corrected transposition who had not undergone cardiac surgery, comparing the echocardiographic findings with those obtained using equilibrium radionuclide ventriculography and gated single-photon emission computed tomography with Technetiumc-99 m sestamibi.

We discovered severe tricuspid regurgitation in 8 patients (61%). Right ventricular ejection fraction, as calculated by nuclear medicine, had a correlation of 0.67 (p = 0.059) with area fractional shortening and 0.84 (p = 0.01) with ejection fraction calculated by the method depending on descent of the tricuspid ring. All patients with severe tricuspid regurgitation also had right ventricular dysfunction. Of these 8 patients, 7 had persistent perfusion defects, while 6 also had ischemic perfusion defects.

Echo contrast had a high sensitivity, at 91%, and also specificity and positive predictive value, both at 100%, for persistent defects, and a negative predictive value of 66% compared to methods depending on nuclear medicine. The sensitivity of contrast echocardiography for detection of ischemic defects was 66%, the specificity 100%, the positive predictive value 100%, and the negative predictive value 77% compared to the methods involving nuclear medicine.

The method depending on descent of the tricuspid ring had the highest correlation with equilibrium radionuclide ventriculography in evaluation of right ventricular function in patients with congenitally corrected transposition.

We conclude that contrast echocardiography is extremely valuable when assessing right ventricular myocardial perfusion, having high sensitivity and specificity for detecting persistent defects, although sensitivity was less for detection of ischemic defects than that of gated single-photon emission computed tomography with Technetium-99 m Sestamibi.

Persistent and ischemic perfusion defects, together with chronic volume overload from tricuspid regurgitation, are the determining factors of right ventricular dysfunction.

We performed a combined Senning and arterial switch operation on a 2-month-old patient with congenitally corrected transposition, Ebstein's malformation producing severe tricuspid regurgitation, ventricular septal defect, pulmonary hypertension, and congestive heart failure. The tricuspid regurgitation was improved. The double switch operation has the advantage of improving the function of the systemic atrioventricular valve, especially in newborns or young infants in whom the outcome of the valvar repair is poor.

Pulmonary arteriovenous fistulas are vascular malformations, which, by virtue of producing abnormal vascular connections proximal to the units of gas exchange, result in intrapulmonary right-to-left shunting. These malformations or fistulas reflect at least in part disordered angiogenesis, and less commonly recruitment and dilation of pre-existing vascular channels.1 Pulmonary arteriovenous fistulas occur in a number of diverse clinical settings. Such fistulas are a well-established feature of the Weber–Osler–Rendu complex, or hereditary haemorrhagic telangiectasia, an autosomal dominant vascular dysplasia characterized by mucocutaneous telangiectasis, epistaxis, gastrointestinal haemorrhage, and arteriovenous malformations in the lung, brain, liver and elsewhere.2,3 They are also seen in the patient with acute or chronic liver disease, disease that is usually but not invariably severe, or those with non-cirrhotic portal hypertension. They may occur as congenital malformations, single or diffuse, large or small in isolation, and when large or extensive enough may result in hypoxaemia, clinical cyanosis, and heart failure.3 Cerebral vascular accidents are also a well-known complication of this disorder.3 An extensive literature has accumulated with regard to the pulmonary arteriovenous fistulas seen in the setting of the Weber–Osler–Rendu complex, and there is considerable information on the genetics, basic biology, clinical findings, complications and therapeutic interventions of these malformations in the setting of this syndrome.4 These issues, however, are not the primary considerations of this review, although some aspects of this fascinating disorder will be discussed later. Rather the focus will be on pulmonary arteriovenous malformations that develop in the setting of cavopulmonary surgery, and their relationship to the pulmonary arteriovenous fistulas occurring in the hepatopulmonary syndrome. The complex tapestry of these overlapping and intersecting clinical observations will be unfolded in the light of their chronology.

Purpose: When there is partially anomalous pulmonary venous connection to the superior caval vein, intracardiac repair alone can result in obstruction. Although the Warden procedure involving translocation of the superior caval vein is commonly performed as an alternative to atriocavoplasty, follow-up of a larger number of patients in the modern era is lacking. We report and discuss the experience of a single institution with the Warden procedure for correction of partially anomalous pulmonary venous connection to the superior caval vein. Methods: Since 1995, all 16 patients presenting with partially anomalous pulmonary venous connection to the superior caval vein underwent the Warden procedure at a mean age of 7.1 ± 4.2 years, with a range from 0.2 to 14.3 years, and a mean weight of 24.7 ± 14.0 kg, with a range from 4.1 to 52.9 kg. There were 9 males and 7 females. In 8 patients, we performed 10 concomitant procedures, including closure of an atrial or ventricular septal defect in 7, and advancement of the aortic arch in the other. Results: There were no deaths, and only one episode of postoperative sinus bradycardia with intermittent junctional rhythm, which resolved spontaneously during temporary atrial pacing. All patients were discharged home in normal sinus rhythm at an average of 4.1 ± 2.2 days after the procedure, with a range from 2 to 10 days. All are currently in the first grade of the New York Heart Association up to 5.6 years postoperatively. There is currently no evidence of sinus nodal dysfunction, nor obstruction of the superior caval vein, in any patient. Conclusion: The Warden procedure for partially anomalous pulmonary venous connection to the superior caval vein produces excellent results, preserves the function of the sinus node, and should be routinely considered for the repair of this lesion.

The technique of deep hypothermia with circulatory arrest has been important in the history of the evolution of cardiac surgery. Wilfred G. Bigelow, working in Toronto in the late 1940s, performed pioneering research on hypothermia, and developed a workable technique of hypothermia in human cardiac surgery.1 Based upon Bigelow's experimental premises, F. John Lewis, at the University of Minnesota, also conducted a number of experiments utilizing hypothermia. On September 2, 1942, Lewis operated on a 5-year-old girl with an atrial septal defect under general hypothermia with inflow occlusion. He was assisted by Richard Varco, Mansur Taufic, and C. Walton Lillehei. Rubberized refrigerated blankets were used to cool the patient to 28°C. The septal defect was closed during five and a half minutes of inflow occlusion. This was the world's first successful open operation on the human heart performed under direct vision, and marked the beginning of the era of open heart surgery. Now, as amazing and as primitive as that methodology may seem, those of you who read Life magazine, or watch the Discovery Channel on television, are aware that, in parts of the Soviet Union, a large fraction of today's open heart surgery is performed not using the technique of cardiopulmonary bypass, but rather using the methodology of immersion hypothermia from the 1950s, with surprisingly good results.

more than 30 years ago, fontan and baudet proposed bypass of a dysfunctional right ventricle by connecting the pulmonary arteries directly to the right atrium, the so-called atriopulmonary anastomosis. since then, much experience has been accrued in the field of the functionally univentricular circulation. the proposed connections have been subjected to several modifications, aiming towards minimizing the losses of energy in the cavopulmonary system, and thereby improving the clinical outcomes. a remarkable improvement was achieved with the introduction of the concept of the total cavopulmonary connection, specifically the combination of a bi-directional glenn anastomosis with a tubular intracardiac extension from the inferior caval venous to the pulmonary arteries. this design was shown to avoid the dissipation of energy associated with the swirling patterns seen in the traditional atrio-pulmonary anastomosis.

Repair of complex malformations that necessitate restoration of continuity between the right ventricle and the pulmonary arteries can now safely be performed with low morbidity and mortality. Major concerns still remain on the long-term outlook for these patients, and about the durability of the different prostheses used to restore that continuity, whether during initial correction or at the time of reintervention for failure of the conduit or pulmonary regurgitation. In this review, we discuss the salient morphologic features of the right ventricular outflow tract, and then focus on the indications for early and late intervention, current therapeutic options, and outcomes.

hypoplasia, with or without dysplasia, of the subpulmonary ventricle is found in association with a wide spectrum of complex congenital cardiac anomalies. in these cardiac defects, the systemic ventricle is usually normal, while the subpulmonary one, usually of right morphology, is incapable of supporting the entire flow of blood to the lungs. there is now an alternative to the more classical functionally univentricular type of repair, which can be achieved by means of the total cavopulmonary connection, namely the partial biventricular, or the so called “one and a half ventricle” repair. in selected cases, by reducing the preload on the subpulmonary ventricle by construction of a bi-directional cavo-pulmonary shunt, it is possible to achieve complete separation of the pulmonary and systemic circulations, whilst still maintaining pulsatile flow of blood to the lungs. in this review, we describe our experience with the “one-and-a-half ” ventricular option, analysing the role of preoperative evaluation of the subpulmonary ventricle, and describing our results over the short and intermediate term.

A quiet and bloodless field providing optimal surgical conditions has been a crucial prerequisite for the performance of complex cardiac repairs in early life. The use of deep hypothermic circulatory arrest has fulfilled this role, and has been a catalyst for the development of neonatal and infant cardiac surgery. The recently increased awareness of possibly increased incidence of adverse neurological events and developmental outcome associated with this technique,1–5 however, has led to a general trend away from its use. In its place, techniques have been developed to provide cerebral perfusion during reconstruction of the aortic arch and the Norwood operation. Some have described the techniques as regional low-flow perfusion. In our opinion, they are described more accurately as antegrade regional cerebral perfusion. In this review, we discuss the recently described techniques for such antegrade regional cerebral perfusion during surgery on the aortic arch, with emphasis both on the Norwood operation and the observed physiological changes in the cerebral and systemic circulations. The neurologic and developmental outcomes following the use of the technique are still unknown.

We describe two cases of an isolated cleft of the mitral valve in transposition with intact ventricular septum. The cleft is positioned leftward in the pulmonary leaflet, at about 2 o’clock, when viewed from below looking at the cardiac short axis with the right ventricle to one’s left hand. Such clefts, when seen in the Taussig-Bing malformation are also positioned leftward.

In keeping with our current knowledge of cardiac development, our cases provide further evidence that transposition with an intact ventricular septum is the end-point of the Taussig-Bing spectrum.

The aim of our study is to provide data on the prevalence of disturbances of rhythm in the general population of children. Accurate estimates of true prevalence of such disturbances of rhythm from large samples are mandatory if we are to interpret properly electrocardiographic abnormalities. We analysed prevalence of disturbances of rhythm in a population of 152,322, comprised of 71,855 elementary school students, 36,692 males and 35,163 females, aged from 5 to 6 years, and 80,467 students of junior high school, 41,842 males and 38,625 females, aged from 12 to 13 years. We analysed the prevalence of premature atrial and ventricular contractions, first, second and third degree atrioventricular block, incomplete and complete right bundle branch block, Wolff-Parkinson-White syndrome, and prolongation of the QT interval. The prevalence of disturbances of rhythm in total rose with age, being found in 1.25% of elementary school students and 2.32% of junior high school students, and was higher in males than females, at 2.00% as opposed to 1.38%, both values being statistically significant at a level of less than 0.0001. Prevalences of all types of rhythmic disturbances were higher in junior high school students than elementary school students (p < 0.0001). Premature atrial and ventricular contractions and prolongation of the QT interval were higher in female than male students, at percentages of 0.089, 0.497, and 0.02 for males, and 0.123, 0.534 and 0.027 in females (p < 0.0001). In contrast, incomplete and complete right bundle branch blocks were higher in males than females, at 0.983% and 0.083% in males versus 0.410% and 0.161% in females (p < 0.0001). Disturbances of rhythm increased with age, and conduction disturbances were higher in male students than female, although premature atrial and ventricular contractions and prolongation of the QT interval were more frequent in female. These data may be useful for future comparative studies of disturbance of rhythm in children.

A patient with tetralogy of Fallot suffered minimal disability until the age of 45 years, when she developed bacterial endocarditis complicated by hemiplegia. She remained well, but became markedly polycythaemic and, at the age of 50 years, underwent surgical correction to reduce the risk of further thromboembolic incidents. She continued in remarkably good health until her death from lobar pneumonia in her 84th year. We present the post-mortem findings, since as far as we are aware this is the longest recorded survivorship of a patient with tetralogy of Fallot.

Acquired lengthening of the QT interval due to hypocalcaemia is a rare cause of arrhythmia in childhood. Early recognition, rapid institution of appropriate cardiac monitoring, and replacement therapy are essential. An endocrinal work-up may be necessary to exclude primary disorders of calcium metabolism. We report four cases documenting the varied clinical spectrum in which hypocalcaemic-induced prolongation of the QT interval and arrhythmia can occur in childhood.

In 1995, Miami Children's Hospital recognized an institutional problem with its programme providing surgical treatment for congenital cardiac malformations. There was a high rate of mortality for neonatal surgery, and no patients had survived attempted first stage palliation for hypoplastic left heart syndrome. The hospital enlisted nationally recognized consultants in congenital cardiac surgery and cardiology to review the existing programme, and to make recommendations for improvement. Based on these recommendations, a new team was recruited. The recruits were a young attending surgeon, an interventional cardiologist, and a cardiac intensivist, attracted from recognized centers of excellence in Boston and Toronto.