Biomedical subjects
Publications and source records attributed to M Payot.
We present a patient with congenital heart disease and haemodynamically poorly tolerated wide QRS tachycardia. Differential diagnosis and therapy are discussed. After the patient underwent heart transplantation, and the substrates for ECG abnormalities and arrhythmias were demonstrated in the explanted heart.
GOAL: To evaluate the impact of the Ross operation, recently (1997) introduced in our unit, for the treatment of patients with congenital aortic valve stenosis. METHODS: The period from January 1997 to December 2000 was compared with the previous 5 years (1992-96). Thirty-seven children (< 16 yrs) and 49 young adults (16-50 yrs) with congenital aortic valve stenosis underwent one of these treatments: percutaneous balloon dilatation (PBD), aortic valve commissurotomy, aortic valve replacement and the Ross operation. The Ross operation was performed in 16 patients, mean age 24.5 yrs (range 9-46 yrs) with a bicuspid stenotic aortic valve, 7/10 adults with calcifications, 2/10 adults with previous aortic valve commissurotomy, 4/6 children with aortic regurgitation following PBD, and 1/6 children who had had a previous aortic valve replacement with a prosthetic valve and aortic root enlargement. RESULTS: PBD was followed by death in two neonates (fibroelastosis); all other children survived PBD. Although there were no deaths, PBD in adults was recently abandoned, owing to unfavourable results. Aortic valve commissurotomy showed good results in children (no deaths). Aortic valve replacement, although associated with good results (no deaths), has been recently abandoned in children in favour of the Ross operation. Over a mean follow-up of 16 months (2-40 months) all patients are asymptomatic following Ross operation, with no echocardiographic evidence of aortic valve regurgitation in 10/16 patients and with trivial regurgitation in 6/16 patients. CONCLUSIONS: The approach now for children and young adults with congenital aortic valve stenosis should be as follows: (1) PBD is the first choice in neonates and infants; (2) Aortic valve commissurotomy is the first choice for children, neonates and infants after failed PBD; (3) The Ross operation is increasingly used in children after failed PBD and in young adults, even with a calcified aortic valve.
OBJECTIVE: A retrospective study to review the experience of a single center with surgery for aortic coarctation over a period of 30 years (1970-1999). METHODS: Criteria for inclusion: (a) aortic coarctation, isolated or associated with congenital heart defect; (b) surgery between 1970 and 1999. Data recorded: (1) date of surgery; (2) age at surgery; (3) associated lesions; (4) surgical technique; (5) simultaneous surgical procedures; (6) early and late surgical results in term of: (a) deaths; (b) need for reoperation because of re-coarctation or other cardiac lesion; (c) residual/recurrent pressure gradient, evaluated at cuff/Doppler at rest; (d) systemic hypertension, requiring medical treatment. RESULTS: One hundred and forty-one patients underwent surgery for aortic coarctation: 30 neonates, 29 infants, 45 children and 37 adults. Associated lesions were found in 8/37 (=21.6%) adults and in 73/104 (=70.1%) pediatric patients. There were no hospital deaths. During the follow-up there were one late death in the adults group (1/37=2.7%) and three late deaths in the pediatric group (3/104=2.9%), all unrelated with aortic coarctation. Re-operation because of re-coarctation occurred only in ten late survivors of the pediatric group (10/101=9.9%), 9/10 operated on before 1980 (P<0.00001). End-to-end anastomosis, enlarged to the aortic arch in neonates, was associated with the lowest incidence of re-coarctation (P<0.005). A significant (>20 mmHg at rest) pressure gradient was found in none of the adults, and in seven of the 91 pediatric patients (7/91=7.7%) late survivors. Three adults (3/36=8.3%) late survivors are on medical treatment to control systemic hypertension. CONCLUSIONS: The long-term results of our retrospective study confirm that surgery has to be considered the gold standard for the treatment of aortic coarctation. The interventional angioplasty techniques have to provide long-term outcome at least similar to the results obtained with surgery.
Type II mucopolysaccharidosis (Hunter's disease) is a hereditary condition due to a deficit of a lysosome specific hydrolase (iduronate sulfatase) inducing an accumulation of dermatane-sulphate and heparane-sulphate in certain organs. Cardiac involvement is constant in this disease and manifests itself essentially by aortic valve stenosis and insufficiency and/or mitral insufficiency which is progressive, irreversible and life-threatening. Two brothers of Portuguese nationality suffering from a so-called slight form of this disease had classical mucopolysaccharide infiltration of their aortic valves. The elder brother, aged 11, had severe aortic insufficiency associated with mild stenosis requiring treatment with vasodilator drugs. The younger, aged 8, had asymptomatic mild aortic regurgitation. Curiously, mitral valve prolapse with regurgitation was present in both children. The association of mitral valve prolapse and type II mucopolysaccharidosis, without other typical cardiac involvement, has only been reported once in the literature.
Determination of the proper length of the tubular prosthesis is a major issue when performing a systemic-pulmonary artery shunt. The procedure is simplified by using a prosthesis with accordionlike properties. This was demonstrated in 7 consecutive infants with complex congenital heart defects, in whom systemic-pulmonary artery shunts were placed without early or late complications.
The authors report the case of a neonate, a premature 36 weeks gestation male child who presented with a spontaneous thrombosis of the juxtaduetal aorta at 3 days of age. The clinical presentation mimicked that of severe coarctation with cardiocirculatory failure. The diagnosis was ineffective, the introduction of low dose alteplase (plasma activator of human recombinant plasminogen) with continued anticoagulation resulted in near complete lysis of the clot and avoided surgery. Thromboembolic cerebral and renal complications were observed during treatment. However, 6 months after the acute episode, there were no clinical or echographic sequellae. Global renal function remained normal despite mild atrophy of the parenchyma of the left kidney. This combined treatment represents an alternative to high risk surgery. The follow-up of this form of management should be rigorous in view of the potential renal and cerebral complications.
Most of the time atrial septal defects (ASD) are sporadic. Familial forms are characterised by the same type of the ASD, and are frequently associated by other cardiac, osteoarticular (Holt-Oram syndrome) or atrioventricular conductions abnormalities (ostium secundum ASD with prolonged PR, sinus venosus ASD with sinusal bradyarrhythmia). This report describes a family in which the father and his two children present an ASD, each one of a different type (ostium primum ASD, ostium secundum ASD, sinus venosus ASD), without any other associated malformations. To the best of our knowledge, this is the first report of a family in which three different ASD types are present.
Explore the source record for details and available documents.
OBJECTIVE: To evaluate the hypothesis that pulmonary venous congestion and oedema manifested predominantly in the right upper lobe in children with mitral regurgitation occur more frequently than previously thought. MATERIALS AND METHODS: Three radiologists retrospectively and independently reviewed the plain chest radiographs of 54 children (26 girls, 28 boys, age range 2 days-18 years, median 9.5 years) with mitral regurgitation admitted to our institution during a 5-year period. Radiographs showing pulmonary venous congestion and oedema manifested predominantly in the right upper lobe were identified. Clinical records of these patients were studied to exclude other causes of pulmonary disease. RESULTS: Radiographic signs of pulmonary venous congestion and oedema were present in all patients, with redistribution of flow and interstitial oedema in 39 patients (72 %) and alveolar oedema in 15 children (28 %). In 12 (22 %) of 54 children, these findings were localised or predominant in the upper lobe of the right lung; none of the children had predominantly left-sided involvement. CONCLUSIONS: Pulmonary venous congestion and oedema involving predominantly the upper lobe of the right lung in children with mitral regurgitation occur more frequently than previously thought. This finding is useful in the differential diagnosis of right upper lobe abnormalities, such as pneumonia.
In October 1992, severe dilatation of the right atrium was detected in a 35 week foetus. The septal leaflet of the tricuspid valve seemed to be displaced distally causing massive tricuspid regurgitation. The diagnosis of Ebstein's anomaly was made and confirmed after birth. Refractory right heart failure occurred at the age of 10 months and the little girl was operated. At surgery, the right atrium was very dilated; the tricuspid valve was normally positioned and was normally constituted. The right atrial wall, partially resected, contained few muscular fibres and showed patchy fibroelastosis. Four years after surgery, the child remains asymptomatic. Dilatation of the right atrium and the tricuspid annulus caused pseudo-displacement of the septal leaflet of the tricuspid valve. This, combined with the importance of tricuspid regurgitation, led to the erroneous diagnosis of Ebstein's anomaly. It is important to differentiate idiopathic right atrial dilatation from Ebstein's anomaly because surgery is much more difficult in the latter case.
A six month old girl with no significant medical history was admitted to hospital for progressive cyanosis of recent onset refractory to oxygen therapy. There were no detectable cardiac murmurs. Chest X-ray showed an irregular left posterior parahilar infiltration. Echocardiography showed dilatation of the left atrium and ventricle but no cardiac malformation. The diagnosis of pulmonary arteriovenous fistula was suspected. Chest CT scan, magnetic resonance imaging and pulmonary angiography demonstrated the arteriovenous fistula in the lower lobe of the left lung. It was much bigger than the appearances of the chest X-ray suggested. After the left lower lobectomy, the cyanosis completely disappeared. Progressive cyanosis refractory to oxygen therapy without any apparent cardiac or pulmonary disease is strongly suggestive of pulmonary arteriovenous fistula. Surgical treatment is curative whereas the spontaneous outcome of this condition may be lethal.
BACKGROUND: Vascular rings are a classical cause of tracheal and esophagus compression. We report the case of such an abnormality in an infant with neurofibromatosis. CASE REPORT: A 1 week-old male infant with a familial neurofibromatosis presented a stridor with severe respiratory distress. A vascular ring was demonstrated and operated on. The stridor persisted after surgery. A postoperative oesophagogram and tracheobronchoscopy showed an irregular compression of the oesophageal lumen, thought to be due to a residual extrinsic compression. Because the postoperative echocardiogram showed an extensive tumoral infiltration of both auricles, it was decided to not operate again the child. The postmortem examination revealed a disseminated neurofibromatosis infiltrating trachea, bronchi and also the wall of esophagus. CONCLUSION: Persisting stridor and oesotracheal compression postoperatively requires search for another cause. Association of vascular ring and neurofibromatosis is probably not fortuitous.
Five days after surgical repair of pectus excavatum, this 7-year-old boy had a right-sided Kirschner wire protruding beneath the skin. The wire was repositioned blindly. Severe congestive heart failure developed. Surgical exploration showed a pierced right atrium, a torn septal leaflet of the tricuspid valve and noncoronary aortic cusp, and a large traumatic ventricular septal defect. The outcome and the indications and possible complications of surgery are discussed.
Eleven newborns admitted consecutively to the neonatal unit with respiratory failure and severe persistent pulmonary hypertension (PPHN) were included in a clinical trial to assess the efficacy of magnesium sulphate (MgSO4) in the treatment of PPHN. A loading dose of 200 mg/kg MgSO4 was given over 20 minutes, followed by a continuous infusion of 20-150 mg/kg/hour to obtain a magnesium blood concentration between 3.5 and 5.5 mmol/l. Mean (SD) duration of treatment was 75.5 (19.8) hours. No other vasodilatory drug was administered before or during the treatment and patients were not hyperventilated. Mean (SEM) PaO2 values significantly increased from 42.6 (8.8) before treatment to 70.3 (24.1) mm Hg after 24 hours, with no change in pH or PCO2. Oxygen index and alveolar-arterial oxygen gradient (A-aDO2) were significantly lower after 24 hours; respectively, 46.8 (15.2) to 28.0 (9.0) and 624.3 (11.3) to 590 (58) mm Hg. Mean airway pressure could be significantly reduced from 19.5 (3.1) to 13.9 (3.9) cm H2O after 72 hours. Mean ventilatory time support was 131 hours and mean total oxygen dependency 10 days. No systemic hypotension nor any other adverse effect were noted. All infants survived and the neurodevelopmental assessment was normal at 6 and 12 months of age. It is concluded that magnesium sulphate is a non-aggressive and low-cost treatment of short duration which is easy to apply. It may have a role in the various treatment of PPHN.
Paroxysmal supraventricular tachycardia is the most frequent significant arrhythmia in the pediatric age group, especially in the first year of life. In neonates and infants there are important limitations for the commonly used drugs such as verapamil and digitalis. In an open Swiss multicentre study we treated 19 children with a total of 29 episodes of tachycardia by means of adenosine i.v. as the drug of first choice. 76% of all the tachycardias were converted, whereas the success rate was 87% if only tachycardias with atrioventricular reentry were considered. The important advantage of adenosine lies in its very short half-life of about 15 seconds, which means that the rare relevant, and the more common mild, side effects are quite limited in duration. A major disadvantage are recurrences in about one third of cases. We conclude that adenosine is an efficient and safe treatment for paroxysmal supraventricular tachycardia in the whole pediatric age group including neonates and infants.
An infant with frequent upper airways infections presented syncopes during meals and weeping since the age of eleven months. Cardiac examination was always normal. At 14 months of age, an echocardiogram with colour Doppler demonstrated a severely stenotic isolated supramitral membrane with severe pulmonary hypertension. The membrane was immediately excised curing the malformation and suppressing definitively the syncopes, probably due to decreased cerebral blood flow during exertion. An echocardiogram should always be performed when syncopes remain unexplained in small children. It allows early diagnosis and treatment of congenital heart defects which do not have auscultatory findings especially those resulting in severe pulmonary venous obstruction.
Two cases of cardiac rhabdomyoma discovered fortuitously at foetal ultrasonography gave rise to no obstructive cardiac signs or arrhythmias either in the antenatal or postnatal periods. These multiple tumours often observed in Bourneville's tuberous sclerosis orientated the clinical investigations to the diagnosis of this disease from the outset. MRI demonstrated cortical tubers and subependymal nodules in both cases. A retinal hamartoma was present in one case. No renal involvement could be detected by ultrasonic examination. No neurological or cardiovascular symptoms appeared during follow-up (20 and 4 months after birth). Investigations in the parents were negative, these two cases being sporadic forms of Bourneville's tuberous sclerosis. The functional prognosis is related to the neurological outcome. Doppler echocardiography would appear to be the best method of following up cardiac rhabdomyomas, and enabled the demonstration of partial regression of the largest tumour in one of these two cases. Cardiac MRI is also an excellent diagnostic tool. As it is usually performed at the same time as cerebral MRI, essential in the follow-up of Bourneville's tuberous sclerosis, it does not represent additional discomfort to the patient.