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[Diaphragmatic paralysis in the postoperative period after heart surgery in children].

OBJECTIVE: The purpose of our study was to determine the incidence, pathological features, clinical relevance and treatment of diaphragmatic paralysis (DP) after pediatric cardiac surgery. PATIENTS AND METHODS: Five hundred fifty-six children who had undergone cardiac surgery between 1990 and 1994 were retrospectively analyzed. Persistent raising of one or both hemidiaphragms on chest x-ray films lead to the diagnosis in all cases. RESULTS: Diaphragmatic paralysis was diagnosed in 13 patients (2.3%) ranging from 3 days to 13 years of age. Atrial septal defect closure (3 cases) and systemic-pulmonary shunt derivations (3 cases) were the type of operations most frequently involved. The other cases included, 2 arterial switch in transposition of the great vessels, 1 repair of total anomalous pulmonary venous drainage, 1 tetralogy of Fallot repair, 1 coarctation and aortic stenosis repair, 1 resection of subpulmonary stenosis of transposition of the great vessels and 1 pacemaker implantation. There was no significant association with the use of extracorporeal circulation. There were seven cases of right hemidiaphragmatic paralysis, but no bilateral paralysis was found. Ipsilateral thoracotomy section concordance was found in all patients and no relation with central venous line placement was found. Diagnosis was made by chest x-ray in all cases, and further confirmation was done using fluoroscopy in 6 patients and ultrasound studies in 2. Prolonged mechanical ventilation was needed in 3 patients, and one patient required diaphragmatic plication. No long term complications were found. CONCLUSIONS: Diaphragmatic paralysis is a rare complication of pediatric cardiac surgery which must be suspected when failed attempts of respiratory weaning, not attributable to cardiac or pulmonary problems, are present.

Adolescent↗

The 'spin' technique: a new method for examination of the fetal outflow tracts using three-dimensional ultrasound.

BACKGROUND AND OBJECTIVE: The prenatal detection of congenital heart defects remains one of the most difficult challenges for the sonologist/sonographer when performing the second- or third-trimester screening examination. The four-chamber view has been used for a number of years as the primary screening image for detection of heart defects, but the inclusion of the right and left outflow tracts increases the detection of cardiac malformations. One of the difficulties, however, is obtaining and interpreting two-dimensional images of the outflow tracts. This paper reviews a new technique using three-dimensional (3D) multiplanar imaging that allows the examiner to identify the outflow tracts within a few minutes of acquiring the 3D volume dataset by rotating the volume dataset around the x- and y-axes. METHODS: 3D multiplanar imaging of the fetal heart using static 3D or spatio-temporal image correlation (STIC) imaging allows the examiner to obtain a volume of data that can be manipulated along the x- and y-axes using reference points from the four-chamber view, five-chamber view, three-vessel view at the level of the bifurcation of the pulmonary arteries, and three-vessel view at the level of the transverse aortic arch and trachea. RESULTS: The full length of the main pulmonary artery, ductus arteriosus, aortic arch and superior vena cava could be identified easily in the normal fetus by rotating the volume dataset along the x- and y-axes. The vessels were identified using the four-chamber view, the five-chamber view, and the two three-vessel views. The technique was useful in identification of d-transposition of the great vessels and evaluation of the outflow tracts in hypoplastic left heart syndrome. CONCLUSION: 3D multiplanar evaluation of the fetal heart allows the examiner to identify the outflow tracts using a simple technique that requires only rotation around x- and y-axes from reference images obtained in a transverse sweep through the fetal chest.

Aorta, Thoracic↗

Discontinuity between the heart and the pulmonary circulation. Its management with the use of a valved external conduit.

The Hancock conduit that contains a porcine xenograft valve has been used in part of the cardiac repair of 22 patients with complex congenital heart disease. Five patients had a severe form of tetralogy of Fallot; six had pulmonary atresia; five had transposition of the great vessels, ventricular septal defect (VSD), and pulmonic stenosis; five had truncus arteriosus; and one had "corrected" transposition, VSD, and pulmonic stenosis. The hospital mortality was 2/22. This conduit has proved a satisfactory method to establish right ventricular-pulmonary artery continuity.

Bioprosthesis↗

[Anesthesia and perioperative complications of correcting transposition of the great arteries using the Jatene technique].

We present a retrospective morbi-mortality study in 12 patients undergoing Jatene's arterial repair for transposition of the great vessels between 1988 and 1992. Half of the patients were boys ranging in age from 4 days to 35 months. In 34% heart failure was grade III, while in 65.6% it was grade IV (NYHA). Anesthetic induction was with ketamine in 11 patients and with halothane in 1. Maintenance was with pancuronium and fentanyl supplemented with N2O in 2 and with isoflurane in 1. The overall intra- and perioperative death rate was 16.6%. Nine cases have undergone surgical repair since 1988 with no mortality. We conclude that Jatene's technique is the best alternative for repair of transposition of the great vessels when there is no hypoplasia of the right cavity.

Anesthesia, General↗

[Functional capacity and rhythm changes after atrial correction of transposition of great arteries].

A group of 12 patients, previously submitted to atrial correction of the transposition of the great vessels, were studied through stress test with direct metabolimetry and Holter to assess aerobic functional capacity and its relationship with conduction disturbances. The results were compared with those obtained with a control group of 23 non athletic healthy children. All the patients showed amputation of their heart rates on effort, though this parameter and the oxygen consumption were normal at low work load levels. The VO2 at anaerobic threshold, the maximum VO2, and the maximum work load were also decreased in most of the patients. Holter studies demonstrated sick sinus syndrome in nine of them. Aerobic functional capacity was reduced in most of the patients with atrial correction of the transposition of the great vessels. The amputation of the heart rate on effort is a common finding though its relationship with the decrease of the aerobic functional capacity in these patients is limited. Finally, the stress test is probably more sensible than Holter for the detection of slight degrees of sinus disfunction.

Adolescent↗

Accessory mitral valve tissue causing left ventricular outflow tract obstruction.

Although left ventricular outflow tract obstruction is commonly associated with congenitally corrected transposition of the great vessels, this obstruction is seldom caused by accessory mitral valve tissue. Three cases in which accessory mitral valve tissue caused left ventricular outflow tract obstruction in children are described. Two had congenitally corrected transposition and one had normally connected great vessels. The accessory leaflet tissue, which was identified by echocardiography and angiography, was attached by chordae tendineae to normally sited papillary muscles and herniated into the left ventricular outflow tract during systole. Operation was successful in these patients. The accessory valve tissue was excised via an arteriotomy in the great vessel that arose from the left ventricle. The obstructive tissue was excised close to its peripheral attachments in the outflow tract and its chordae tendineae were divided. Resection was performed without injury to the abnormally placed conduction system or to the normal valve structures.

Adolescent↗

Echocardiographic considerations in demonstrating complex anatomy of criss-cross atrioventricular valves and discordant atrioventricular and ventriculoarterial relations.

The associated features of 1-looping of the ventricles (ventricular inversion) with D-transposition of the great vessels and criss-crossing atrioventricular valves are an unusual constellation of cardiac anomalies resulting in corrected transposition physiology. The following case presentation depicts the fetal and postnatal considerations of two-dimensional and color Doppler echocardiography in accurately detailing the complex intracardiac connections associated with this arrangement and also warns of the potential pitfalls in echocardiographic assessment.

Echocardiography↗

[Univentricular heart with left morphology with permeable atrio-ventricular valves. Study of the bulbo-ventricular foramen with color echo-Doppler. Importance for the surgical repair].

INTRODUCTION AND OBJECTIVES: Bidimensional and color-coded Doppler echocardiography were used to study 19 patients with univentricular heart, permeable atrioventricular valves, muscular bulbo-ventricular foramen and an anterior rudimentary chamber. We evaluated the influence of the foramen upon the size of the great vessels, and in consequence the most appropriated surgical techniques. MATERIAL AND METHODS: The foramen was measured in two projections at the end of the systolic period, and its area was calculated, according to the body surface, with the formula S = pi (D1/2 x D2/2). Indexes lower than 2 cm2/m2 were considered as restrictive. We studied 19 patients that presented a mean age of 11 +/- 12 years (range 1 day-39 years). The patients were classified in two groups. Group A: patients who had transposition of the great vessels (13 cases). Group B: patients who had normal connection of the great vessels (6 cases). Each group was classified in two subgroups: subgroup 1, patients who presented a restrictive foramen, and subgroup 2, patients who presented a normal foramen. In this study, we evaluated the associated cardiac anomalies. RESULTS: Group A. Thirteen cases. The index value for the whole group was 2.47 +/- 1.18 cm2/m2. The first subgroup included 6 cases with a restrictive foramen, 2 patients presented pathology of the arch. Four patients had a pressure gradient between the ventricle and the rudimentary chamber beyond 20 mmHg. The index of this subgroup was 1.68 +/- 0.39 cm2/m2, the median was 1.7. Seven cases, with a normal foramen, were included in the second subgroup, six of them had pulmonary valve stenosis, and the other one had undergone a pulmonary artery bandage 2 months before. Only one patient had a gradient pressure at the foramen beyond 20 mmHg. The index for this second subgroup was 3.34 +/- 0.83 cm2/m2. GROUP B: Six patients with normal connection of the great vessels were included. The index was 1.56 +/- 1.17 cm2/m2. The first subgroup included 3 cases with a restrictive foramen. The index was 0.72 +/- 0.29 cm2/m2 (range 0.41-1) and the median was 0.75. Pressure gradient between the ventricle and the rudimentary chamber was greater than 20 mmHg. Three patients with a normal foramen were in the second subgroup and here, the index was under normal limits 2.4 +/- 0.52 cm2/m2 (range 2.1-3), the median was 2.1. One patient underwent a pulmonary artery bandage two months before. CONCLUSIONS: Bulbo-ventricular foramen size/body surface index may exert an influence on the size of the vessel connected to the rudimentary chamber. The index vary in a negative way with the evolution time, or in those cases with transposition of the great vessels after a pulmonary artery bandage. Whenever the foramen is restrictive, the pulmonary artery or the aorta have a smaller diameter and the pressure gradient between the ventricle and the rudimentary chamber rises.

Adolescent↗

[Epidemiological and genetic study of 3 congenital cardiopathies with neonatal disclosure].

A familial study was undertaken on 382 probands with transposition of the great vessels, 348 with coarctation of the aorta and 143 with hypoplastic left heart syndrome. It allowed to assess in sibs the frequency of identical congenital heart diseases (respectively 0.15, 0.42 and 3.41%) and of all 7.32%). The epidemiological study did not provide further data worth noting. At the origin of the transposition of the great vessels, tetratogenetic factors are added to genetic factors, whose part is of little importance; hypoplastic left heart syndromes are heterogeneous (recessive autosomal transmission, chromosome abnormalities, sporadic cases). Coarctations of the aorta, which are dependent on different mechanisms are also heterogeneous. It is possible that common factors, whether hereditary or not, play a part on one hand in hypoplastic left heart syndromes and coarctations of the aorta, and, on the other hand, in some congenital heart diseases.

Abortion, Spontaneous↗

Anatomic repair of Taussig-Bing hearts.

From 1978 through 1990, 27 consecutive patients underwent anatomic repair of Taussig-Bing heart (TBH) malformation. Group 1 (seven patients) underwent intraventricular repair by rerouting of the left ventricular blood stream through a tunnel patch from the ventricular septal defect (VSD) to the aorta. Group 2 (20 patients) underwent an arterial switch with VSD closure. Indications for each type of repair were based on the following criteria: when the great vessel relation is more or less anteroposterior, repair is performed at the arterial level; when side by side, the minimal tricuspid pulmonary valve distance is evaluated; if less than aortic valve diameter or if there are abnormal tricuspid chordae, repair is performed at the arterial level; if greater, pulmonary artery banding is performed in order to wait until suitable weight for intraventricular repair. The mean age at operation was 13.2 months. Coarctation of the aorta was present in 12 patients, abnormal tricuspid chords in eight, and subaortic stenosis in seven. The great vessels were in D-transposition in 12 patients, and they were side by side in 15. Seventeen patients underwent previous palliative surgery. Early (two patients, 7.4%) and late (two patients, 8%) mortality rates were related in group 1 to subaortic stenosis and in group 2 to myocardial infarction. Mean follow-up of 40 +/- 24 months was achieved in 22 survivors. They were all in New York Heart Association (NYHA) class I, without medication. Actuarial survival and freedom from reoperation rates were, respectively, at 5 years 73 +/- 14.6% and 58 +/- 13% (70% confidence limits [CL]).(ABSTRACT TRUNCATED AT 250 WORDS)

Actuarial Analysis↗

Electroejaculation of the paraplegic male followed by pregnancy.

A method is described for the production of an ejaculate the paraplegic male by electrical stimulation from a probe placed in the rectum. A case is reported in which an ejaculate produced by this method was used to inseminate the wife of a paraplegic patient. Pregnancy resulted, but the infant died. Post-mortem examination revealed the presence of transposition of the great vessels.

Adult↗

Bronchopulmonary circulation in d-transposition of the great arteries: possible role in genesis of accelerated pulmonary vascular disease.

Hemodynamic and angiograhic data from 209 patients with d-transposition of the great arteries were reviewed to estimate the incidence of prominent bronchopulmonary circulation and to explore its role in the genesis of accelerated pulmonary vascular disease in these patients. The degree of bronchopulmonary circulation was assessed visually by considering the extent of the pulmonary arterial opacification and the circulation to the left atrium. An initial survey study revealed a marked degree of collateral circulation in 20 of 138 patients with d-transposition having cardiac catheterization before age 2 years at the Hospital for Sick Children, Toronto, between 1967 and 1972. Detailed analysis of 71 additional patients with d-transposition aged 1 week to 72 months (mean 17 months) studied at Children's Memorial Hospital, Chicago, between 1967 and 1974 showed collateral circulation of marked degree in 23 and of mild degree in 14. The bronchopulmonary collateral vessels were more freqently demonstrated in the patients with intact ventricular septum than in those with ventricular septal defect or left ventricular outflow tract stenosis. In a prospective study in 12 of 15 patients during cardiac catheterization the functional patency of the bronchopulmonary collateral circulation was demonstrated by obstructing pulmonary blood flow in the right or left pulmonary artery, or both, with an inflated balloon and obtaining from the pulmonary arterial segment distal to the occlusion blood with an oxygen saturation similar to that of the aorta. A hypothesis is presented concerning the role of systemic hypoxemia and local pulmonary hypoxemia induced by way of the bronchopulmonary collateral vessels and the bronchial arterial vasovasorum in promoting pulmonary vasoconstriction. It is suggested that increased pulmonary blood flow and pressure due to the physiologic features of ventricular septal defect, patent ductus arteriosus or transposition of the great vessels, in the face of this regionally increased hypoxemia results in accelerated pulmonary vascular disease.

Aortography↗

Neural crest cells contribute to normal aorticopulmonary septation.

By analyzing the hearts of quail-chick chimeras, it was found that neural crest cells at the level of occipital somites 1 to 3 migrate to the region of the aorticopulmonary septum. Bilateral removal of this neural crest population prior to migration causes malformation of the aorticopulmonary septum resulting in common arterial outflow channels or transposition of the great vessels.

Animals↗

[Partial distortion of the great vessels in dextroposition and intrapulmonary continuity (Taussig-Bing variety)].

A case of partial distortion of the great arteries in a patient with dextroposition and mitropulmonary continuity is described (Taussig-Bing variety). The embryogenesis and anatomy in comparison with others similar cases found in the literature is analyzed and discussed. The cardiac defect is a partial distortion of the great arteries and its analysis permit to conclude that the conal muscle is not an important anatomic feature; its presence does not play a important roll in the genesis of the heart malformation. This case is compared with the so called "transposition of the great arteries with posterior aorta" and it is concluded that these heart defects have the same embryogenesis and that the relation of the great arteries themselves and with the ventricles depend of different grades of incorporation of the conus. It can be said also that this group of cardiac defects are not true transpositions of the great vessels, because the aorta does not arise anteriorly to the crista supraventricularis.

Adult↗