Unusual form of coarctation of the distal thoracic aorta.

We report the case of a 3-month-old girl with a rare form of coarctation involving the lower descending thoracic aorta. Because of clinical findings of congestive heart failure and hypertension, early repair was recommended. Surgical intervention in young patients with this unusual localization presents a complex challenge. Aortic reconstruction was carried out by patching the stenotic segment with autologous arterial tissue. Three years after the repair, there is no evidence of recoarctation or aneurysmal dilation.

Transesophageal echocardiography with the use of a four-millimeter probe.

Transesophageal echocardiography with the use of pediatric probes is nowadays commonly performed. However, in small children, insertion of a probe with a diameter of 7 mm may be traumatic or even impossible. We therefore tested a 17-element, 4 mm transverse plane probe in 136 pediatric patients, mainly in the operation room, catheterization laboratory, or the intensive care unit, and in three healthy adult volunteers. This probe was easy to insert, particularly during emergency situations, did not cause any complication in any patient, and provided satisfactory information despite the low number of elements. The use of a 4 mm transesophageal probe can improve the management of neonates with congenital heart disease in the operating room or the neonatal intensive care unit.

Usefulness of biplane transesophageal echocardiography in neonates, infants and children with congenital heart disease.

A study was performed to assess the feasibility, additional diagnostic value and potential applications of biplane transesophageal echocardiography in neonates, infants and children. One hundred thirty-two consecutive studies were attempted in 111 anesthetized children with congenital heart disease. Longitudinal and transverse planes were compared using 3 methods: (1) separate 7 mm longitudinal and transverse pediatric transducers used sequentially; (2) an experimental 9 x 8 mm biplane pediatric transducer; and (3) a standard adult biplane transducer (12 x 9 or 13 x 9 mm). In all but 1 patient, a probe could be inserted. The longitudinal plane provided superior visualization of both the right and left ventricular outflow tracts, the interatrial septum, the main pulmonary artery, the ascending aorta and the right coronary artery. In 18 patients (16%), the longitudinal plane provided completely new diagnostic information that was not obtained with combined transthoracic and transverse plane transesophageal echocardiography. However, the transverse plane was mandatory for demonstration of the 4-chamber view, short-axis cross sections through the great arteries, the distal right pulmonary artery and bifurcation of the left coronary artery. The longitudinal plane is complementary to the transverse plane, but cannot substitute for it.

Valve morphology in complete atrioventricular septal defect: variability relevant to operation.

The remodeling of the atrioventricular valves in patients with complete atrioventricular septal defects is the crucial part of surgical repair. Variability in valve morphology is an important factor. This study evaluates the variability in morphology of the anterior and posterior leaflets in 30 heart specimens. All hearts had an anterior bridging leaflet: Rastelli type A in 12, type B in 2, and type C in 16. The posterior leaflet revealed four morphologic patterns: a right- and left-sided posterior leaflet, both inserting directly onto the crest of the ventricular septum (5 hearts); a common posterior leaflet attached to the septal crest by a membrane (2 hearts); a common posterior leaflet attached to the septal crest by multiple chordae (13 hearts); and a virtually free-floating posterior leaflet (11 hearts). The categorization is surgically relevant in making a distinction between hearts with and without an interventricular communication underneath the posterior leaflet. Surgically relevant variations occurred also in arrangement and positioning of chordae originating from the right septal side. There was no relationship between the Rastelli classification of the anterior leaflet and that of the posterior leaflet. The variability in morphology of the posterior leaflet and its attachments to the ventricular septum appear equally crucial for successful repair as that of the anterior leaflet.

Is there an anatomic basis for subvalvular right ventricular outflow tract obstruction after an arterial switch repair for complete transposition? A morphometric study and review.

The study was initiated by reports on right ventricular outflow tract obstruction in complete transposition of the great arteries after an arterial switch repair. We investigated 39 heart specimens with native, unoperated transposition of the great arteries. Of these, 14 hearts had a ventricular septal defect; 25 had an intact ventricular septum. In each heart specimen the narrowest site of the subaortic outflow tract was measured and compared with the circumference of the aortic orifice. Obstruction was considered to be present if the outflow tract circumference was less than that of the aortic orifice. In addition, the diameter of the ascending aorta immediately above the level of the valve orifices was measured and compared with that of the pulmonary trunk. An obstruction was present in the subaortic right ventricular outflow tract of two hearts (5.1%): one of the obstructions, in a neonatal heart with intact ventricular septum, was caused by a prominent supraventricular crest and anterior trabeculations; the other obstruction was an additional extensive muscular hypertrophy, in the heart of a 13-year-old patient with a similar anatomy, and a septal defect. A mismatch between the diameters of the ascending aorta and the pulmonary trunk was present in 15 of 32 hearts measured. Our observations and a review of the literature confirm that subvalvular right ventricular outflow tract obstruction in hearts with native transposition of the great arteries is infrequent. Nevertheless, the anatomic characteristics of the right ventricular outflow tract are such that the tract is intrinsically narrow and muscular hypertrophy may easily lead to obstruction. After an arterial switch operation, subvalvular obstruction could be caused by dynamic processes analogous to those observed after relief of isolated pulmonary valve stenosis. Anatomic subvalvular obstruction could be due to either an obstruction that was not identified before operation or (a purely speculative hypothesis) subtle degrees of mismatch in size between the proximal aorta and the pulmonary trunk, which may be considered irrelevant at time of operation but may also set into pace a process of ongoing adaptive infundibular hypertrophy.

Total anomalous pulmonary venous connection.

Repair of total anomalous pulmonary venous connection was performed on 31 patients aged 12 days to 14 years (18 less than 6 months). The connection type was supracardiac in 20 cases, cardiac in nine, infracardiac in one case and mixed in one. Deep hypothermia and circulatory arrest were used in 23 cases (74%). In supracardiac type cases the atrial septal defect was closed through the left atriotomy, without enlargement of the left atrium. Extubation in the operating room was possible in 26 cases (84%). Three patients (9.6%) died, one (with connection to the coronary sinus) soon after operation, due to a management error, another (with connection to the right superior vena cava) of pulmonary edema, and an infant with mixed-type connection 1 week postoperatively, presumably from an arrhythmia. No patient required reoperation because of late pulmonary venous stenosis. There were no late deaths. The technique of elevating the cardiac apex provided excellent exposure in the supracardiac and infracardiac types. Progressively earlier referral during the study period facilitated prompt operation and improved patient salvage.

Transesophageal echocardiography in pediatric patients: preliminary results.

Transesophageal echocardiography was attempted in 59 pediatric patients with congenital heart disease in the operating room (n = 33) or during heart catheterization or in the intensive care unit (n = 26). Six different commercially available transducers were used with diameters ranging from 7 to 15 mm. Age ranged from 1 day to 16 years, and body weight ranged from 3.7 to 65 kg. Objectives of the study were to determine (1) minimum body weight in which transesophageal echocardiography with various probes is possible, (2) additional diagnostic value, and (3) potential applications. In three cases (one surgical and two nonsurgical) the probe could not be inserted. Minimum body weight was 17 to 20 kg for probes with a diameter of greater than or equal to 13 mm and approximately 12 kg for the 11 mm probes. A 7 mm probe, on the other hand, could be inserted easily in all patients (including a neonate) in whom transesophageal echocardiography was attempted. In 11 of 56 patients, additional diagnostic information was obtained. Thus, transesophageal echocardiography is feasible in the pediatric age group provided that special probes are used in small children. Additional diagnostic information can be obtained, and the technique is of value during cardiac surgery or balloon interventions for evaluation of the efficacy of the procedure and for monitoring ventricular function.

Anomalous origin of the left coronary artery from the pulmonary artery: repair by aortic reimplantation.

From 1980 to 1990, 12 patients (mean age 2.5 years, range 5 months to 9 years) with anomalous origin of the left coronary artery from the pulmonary artery were treated surgically. Five infants were operated upon in the first year of life because of persistent symptoms of congestive heart failure. In all cases, a two-coronary system was constructed by direct aortic reimplantation of the anomalous vessel with no deaths early or late over a follow-up period of up to 10 years. The technique of reimplantation was facilitated by transection of the main pulmonary artery. One patient with severe mitral regurgitation underwent, in addition, a mitral annuloplasty. A pulmonary valvotomy was performed in another patient with associated pulmonary stenosis. Three patients are receiving medication. The reimplanted anomalous left coronary artery was patent in each reevaluated patient (10/12). Left ventricular function improved considerably in all cases. Patients with symptoms should undergo repair soon after diagnosis. Direct aortic reimplantation should be technically feasible in even the smallest infant. Operative mortality is related to preoperative conditions and severity of ischemic damage of the myocardium.

Tetralogy of Fallot with subpulmonary ventricular septal defect.

Among a total of 608 patients with tetralogy of Fallot, 35 with tetralogy and subpulmonary ventricular septal defect (VSD) have undergone intracardiac repair at the Children's Hospital, Buenos Aires. The crista supraventricular was absent in 62.9% of these patients; it was hypoplastic in 37.1%. Only 3 patients (8.6%) were severely cyanotic and required operation in infancy. The main and left and right pulmonary arteries were relatively large in all but 1 patient. Nevertheless, patch-graft enlargement across the pulmonary valve ring was necessary in 20 (57.1%). We believe that closure of the subpulmonary VSD increase the apparent severity of the pulmonary stenosis and necessitates patch-graft enlargement in patients in whom the appearance of the right ventricular outflow tract before repair would not have suggested its need.

Supravalvular mitral stenosis associated with ventricular septal defect.

We describe three patients with supravalvular mitral stenosis caused by a diaphragm located just above the mitral valve and associated with a ventricular septal defect. Two of our three patients also had an associated coarctation of the aorta. The physical signs, ECGs, chest roentgenograms, and cardiac catheterization data are described. Points helpful in the differential diagnosis of this malformation are emphasized. All three patients were treated successfully by surgery.

Anatomic correction of transposition of the great arteries.

Complete anatomic correction of transposition of the great arteries (TGA) and ventricular septal defect (VSD) was performed on 2 infants with high pulmonary arterial resistance. Both patients were operated upon under deep surface-induced hypothermia and limited cardiopulmonary bypass. Direct repair of the anomaly was accomplished by switching the aorta and the pulmonary artery with reattachment of the coronary arteries. The clinical result in the first patient was satisfactory. The postoperative cardiac catheterization and angiogram demonstrated no gradient across either outflow tracts and normal arrangement of the great vessels. The second patient died in the operating room, probably owing to compression or kinking of the left coronary artery.

[Interventricular communications with aortic insufficiency surgical considerations]. / Les communications interventriculaires avec insuffisance aortique. Considérations chirurgicales

The authors report a series of 11 patients with ventricular septal defect associated with aortic incompetence who underwent surgery between 1963 and March 1976. Separate consideration is given to the operations performed before and after 1972. 5 patients were operated on during the first period, with only mediocre results. At this time, the technique of valvuloplasty did not appear to be the right one. During the second period, 6 patients were operated on. In five of them the technique of Plauth, Frater, Spencer and Trusler was used. All these patients have a satisfactory result. The last of the series had an abnormally low commissure, and the adjacent valves were protuberant, and thickened, so that a valvular replacement was carried out. The operation of choice for a ventricular septal defect associated with aortic incompetence is valvuloplasty with closure of the defect. The following criteria are necessary indications for this type of surgery: --the aortic incompetence must be secondary to the prolapse of a valve; --the aortic valve must have three cusps. An early operation makes the valvuloplasty easier, and avoids the problems of damage to the left ventricle from a persistant and progressive aortic incompetence.

[Long-term clinical results with monocomponent insulin (MC lente) in infantile and juvenile diabetes]. / Risultati clinici a lungo termine nel diabete infanto-giovanile trattato con insulina monocomponente (lenta MC)

The clinical role of insulin-antibody formation, with reference to the monocomponent insulin treatment (MC), is discussed in a series of diabetological conditions. On the basis of a five-year-experience, personal results with a MC Lente treatment are presented in 32 cases of juvenile diabetes subdivided as follows: 3 cases with insulin allergy, 5 cases with insulin lipoatrophy, 13 cases with high insulin requirement, 4 cases with brittle diabetes, 7 cases with diabetic microangiopathy (retinal and, or renal). The circulating antibody level was estimated by IgG-Insulin-Binding Capacity (IB), according to Christiansen. After transfer from conventional to MC insulin treatment it was observed: -- disappearance of allergy and total remission of lipoatrophy, in parallel with a reduction of IB titer; -- decrease in insulin requirement and stabilisation of labile diabetic control, not always in concomitance with IB reduction; -- deterioration of advanced diabetic retinopathy and, or nephropathy in spite of IB reduction. It is concluded that MC insulin constitutes a major tool in the treatment of the above mentioned diabetic conditions, except for advanced microangiopathy. Thus a MC insulin treatment should be started, as a rule, in newly diagnosed diabetics, to possibly prevent such complications. However further development of insulin purification techniques, with removal of residual pro-insulin antigenic sites, is to be considered.