Surgical correction of simple transposition of the great arteries by arterial detransposition can only be considered if the left ventricle is capable of functioning under systemic conditions. This is possible at birth but the operation is too big for it to succeed. After a few weeks the left ventricle may lose this capacity but it can be induced to redevelop if it is given a systolic stress by pulmonary banding. This was attempted initially in 30 children aged from 5 weeks to 3,5 years old (20 aged less than 2 months) but had to be abandoned for the following reasons: 8 immediate failures due to severe hypoxia and acidemia, 7 left ventricular failures, mainly in 3 children operated after 6 months of age; 7 severe hypoxemias, 5 pulmonary arterial lesions due to banding and/or the systemo-pulmonary anastomosis associated in some cases; in all, there were 8 failures out of the 17 anatomical corrections performed to date in 22 survivors of banding. The criteria of left ventricular readaptation after banding were difficult to determine. The final result did not appear to be related to either the duration of banding, the degree of aortic desaturation, the ratio of ventricular pressures, or to the various echocardiographic indices. Only the initial hypoxemia (less than 50 p. 100 saturation) with respect to the risk of banding, and the age at which banding was performed (over 6 months) with respect to the risk of anatomical correction, appeared to be obvious risk factors. This is the reason for a new protocol at present under study to try to maintain left ventricular growth by banding in the first days of life, so to ensure a more harmonious and effective, and also less dangerous preparation for anatomical correction. Six new born children have been "prepared" in this way: three had adequate loose banding and underwent atrial correction. Very good results were obtained in the other three patients who have already undergone detransposition at 2,5 and 3 months with excellent immediate results. We believe that neonatal pulmonary banding (before 5 days) when aortic saturation after atrioseptostomy is over 50 p. 100, provides a real alternative to the classical methods of treating simple transposition of the great arteries (Mustard or Senning). Anatomical correction can then be carried out a few weeks later under good conditions.

Angiocardiography↗

PubMed1983

[Infection of an anastomosis: a formidable complication of operations for aortic coarctation].

One 12 day newborn, a 2 year old infant and two children aged 10 and 14, operated for isthmic coarctation of the aorta by three different surgical techniques (resection-anastomosis, angioplasty with an autograft, prosthetic graft) suffered infection of the operative field, resulting in septicemia (3 staphylococcal and 1 yeast infections). The aorta ruptured after 4 days, before any collection of pus in the newborn but a mycotic false aneurysm developed in the three other patients: this was the cause of death by aortic rupture during the 5th postoperative week in one case but was diagnosed and operated successfully in the other two, 13 and 54 days after their first operation. These complications are well known. Therefore, these patients should be closely followed up for at least one month. The occurrence of pyrexia, even slight or late, should be treated with suspicion. Characteristic dilatation of the part of the aorta operated on should be actively looked for by simple radiography, 2D echocardiography and, when in doubt, right heart angiocardiography. The diagnosis is a surgical emergency: the safest technique consists in initially establishing an aorto-aortic deviation by a right sided approach followed by exclusion of the lesion. Surgery is the only means of preventing catastrophic rupture of the aorta.

Adolescent↗

PubMed1982

[Treatment of severe aortic stenosis in infants by transventricular valvotomy].

Explore the source record for details and available documents.

Angiography↗

PubMedDate not supplied

Heterozygous mutation in the pore of potassium channel gene KvLQT1 causes an apparently normal phenotype in long QT syndrome.

Mutations in KvLQT1, a gene encoding a potassium channel, cause both the recessive Jervell and Lange-Nielsen (JLN) syndrome and the dominant Romano-Ward (RW) syndrome. These diseases are characterised by a prolonged QT interval on the ECG, syncopes and sudden death due to cardiac arrhythmias. The JLN syndrome is also associated with a congenital bilateral deafness. We report here a novel missense mutation, W305S, in the pore region of KvLQT1 identified by PCR-SSCP analysis in two consanguineous JLN families. In contrast to several missense mutations found in the same region of KvLQT1 in RW patients which are associated with severe cardiac phenotypes, the W305S mutation is responsible for an apparently normal phenotype in heterozygous JLN carriers.

Consanguinity↗

PubMedDate not supplied

Prenatal ultrasound may predict fetal response to therapy in non-hydropic fetuses with supraventricular tachycardia.

OBJECTIVE: To study the fetal response to prenatal therapy in non-hydropic fetuses with supraventricular tachycardia (SVT) as a function of fetal haemodynamic status at presentation. STUDY DESIGN: Retrospective study. MATERIAL AND METHODS: Between 1990 and 2000, 40 non-hydropic fetuses presented with SVT. Twenty-eight had reciprocating SVT and 12 had atrial flutter. Ten fetuses had significant tricuspid valve regurgitation. All fetuses were treated prenatally. The main outcome measurement was fetal response to therapy as assessed by the rate of prenatal SVT reduction and by the mean time interval to sinus rhythm restoration. RESULTS: The mean gestational age at presentation was 29 +/- 4.9 weeks. Overall, there were 39 live births and 1 intrauterine death. Reduction of SVT was achieved prenatally in 32 cases (80%). Among the 30 cases without tricuspid regurgitation, prenatal conversion to sinus rhythm was achieved in 27 cases (90%) with a mean time interval of 7 days. Among the 10 fetuses presenting with tricuspid regurgitation, the rate of prenatal conversion was significantly lower (5/10) and the mean time interval to conversion was significantly longer (24 days; p = 0.04, Mann-Whitney test). In the subgroup treated by digoxin as first-line therapy (n = 32), the interval to sinus rhythm restoration was also significantly higher in the presence of tricuspid regurgitation, with a slightly but not significantly lower reduction rate. CONCLUSION: The response to prenatal therapy may be poorer in cases presenting with tricuspid regurgitation.

Amiodarone↗

PubMedDate not supplied

[Treatment of critical pulmonary valvular stenoses in newborn infants using percutaneous valvuloplasty. Study of 28 cases].

Twenty-eight neonates aged 1 to 21 days with critical pulmonic stenosis were treated by percutaneous valvotomy consisting of balloon dilatation of the stenotic valve by percutaneous catheterization. The right ventricular systolic pressure (RVP) was either the same or higher than the systemic pressure in all cases but 3. In those it was somewhat lower (80%) than the left ventricular systolic pressure (LVP) so that, on the whole, the RVP/LVP ratio, here called rP ranged from 0.8 to 2 (m = 1.41 +/- 0.36). The procedure failed 5 times because of failure to introduce the balloon through the valve (4 cases) or because of an accident (1 case). All failures occurred during the initial phase of our experience with this procedure as the last 20 trials were successful. The procedure was inefficacious in 3 cases (13%) but immediately successful in the other 20 (87%) with a drop in the mean rP to 0.59 +/- 0.17. During the follow-up period, one neonate continued to present with an important degree of cyanosis, due to a poor right ventricular compliance. He had to be operated on. Pulmonic stenosis reappeared in 6 cases who underwent for a second time a percutaneous valvotomy. The procedure was successful in 3 and failed in 3 who eventually required surgery. Overall, the satisfactory results observed after the procedure persisted over time in 13 cases. In conclusion, interventional catheterization has become almost always possible in neonates with critical pulmonic stenosis. If performed under vigorously controlled conditions including an infusion of prostaglandin E1 in cases with severe cyanosis and the use of large diameter balloons, this method is relatively safe and generally efficacious since in 70% of cases it circumvents the need for surgery.

Angioplasty, Balloon↗

PubMedDate not supplied

[Clinical evaluation and treatment of rhythm and conduction disorders in the fetus].

Fetal echocardiography, including simultaneous and ventricular M mode scans, allows the diagnosis of arrhythmias by studying the relationships between atrial and ventricular contractions. It also assesses cardiovascular tolerance by looking at pericardial or peritoneal effusion, left ventricular contraction and by measuring blood velocity in systole and diastole in umbilical arteries by Doppler techniques. We report here our experience on 221 arrhythmias found out of 1344 fetal echocardiographies performed since 1983. Bradycardia without atrio-ventricular dissociation in otherwise healthy fetuses were excluded since they are physiologic when moderate and transient. In addition, 180 out of our 221 cases had transient premature beats and were also excluded. It remained thus only 41 cases with true significant abnormalities of the fetal rhythm or conduction. Bradycardia with atrio-ventricular block and low ventricular rate was found in 15 fetuses. Ventricular rate was the first risk factor, since the tolerance was poor under 60 beats per minute (b/m). In this group, 9 cases were associated with cardiac malformations: mainly atrio-ventricular discordance, atrio-ventricular canal, double inlet ventricle. In the 6 other, the heart was normal and in only one, an immunologic disorder was found in the mother. Cardiovascular tolerance and gestation age were the two determinants for deciding on early delivery. In all cases, the newborn was referred to a pediatric cardiology center where specific measures (artificial pacing or simple follow-up) were undertaken.(ABSTRACT TRUNCATED AT 250 WORDS)

Amiodarone↗

PubMedDate not supplied

[The socioprofessional rehabilitation of patients with cardiac pacemakers].

Explore the source record for details and available documents.

Adult↗