Electrochemotherapy with intravenous bleomycin in the local treatment of skin melanoma metastases.

BACKGROUND: Electrochemotherapy (ECT) combines chemotherapy and electroporation to increase drug uptake. Its role in cutaneous melanoma metastasis treatment is not well defined; indeed, few studies have been reported, without complete follow-up data. AIM: To prospectively evaluate clinical activity and tolerability of ECT with i.v. bleomycin, and to analyze the response increase associated to repeated sessions, in the largest series of cutaneous melanoma metastases reported to date (n = 233). PATIENTS AND METHODS: 14 stage III relapsed/refractory patients were enrolled according to European Standard Operating Procedures of Electrochemotherapy (ESOPE) guidelines and treated under general sedation using the Cliniporator(TM) pulse generator. RESULTS: A response was obtained in 13/14 patients (93%) after the first ECT, with a complete regression (CR) in 7 (50%). Seven patients underwent a second and three a third ECT on newly appearing and residual lesions, all achieving a response. Overall, a response was obtained in 93% metastases, with lower response rates in >1 cm(2) lesions. The CR rate was 58%; none of the CR nodules relapsed. The repeated ECT sessions gave rise to a new response in 21/29 (72%) re-treated lesions. The local tumor control rate was 74.5% at 2 years. CONCLUSION: ECT is a safe procedure, easily performed in terms of toxicities and cost-effectiveness ratios, and constitutes a therapeutic tool for relapsed/refractory cutaneous melanoma patients. The repeated ECT sessions are associated with a response increase in re-treated lesions which could allow to overcome the reduced activity in >1 cm(2) sized metastases.

Coronary arteriography and atrial thrombosis in mitral valve disease.

The arteriographic findings of neovascularity and fistula formation from the coronary arteries to the left atrium have occasionally been reported in association with atrial thrombosis in patients with mitral valve disease. To establish the diagnostic value of these findings, the preoperative coronary angiograms of 507 patients who underwent open mitral valve surgery were reviewed. Atrial thrombosis was present in 76 patients (14.9 percent). In the 30 patients with angiographic neovascularity and fistula formation, the thrombi were always observed to arise from the circumflex coronary artery. None of these 30 patients had atherosclerotic coronary lesions. In 25 of these patients an atrial thrombus was found at operation. These coronary arteriographic findings, in this selected group of patients, had a predictive accuracy of 83.3 percent, a specificity of 98.8 percent and a sensitivity of 32.8 percent for the diagnosis of the presence of thrombus in the left atrium. No relation was found between these signs and the size and histologic age of the thrombi examined.

Efficacy and safety of indomethacin therapy for polyhydramnios.

The maternal and perinatal outcome of seven gravidas receiving 2.2-2.5 mg/kg per day of indomethacin for polyhydramnios are reported. Such therapy was started between 26 and 33 weeks of gestational age (mean, 30.4 weeks) and lasted for 20.1 days (range, 2-37 days). Median of amniotic fluid index ranged from 47 at the start of therapy (range, 32-53) to 15 (range, 2-50) when indomethacin was ended. Interval between the end of the therapy and the delivery ranged from 0 to 45 days (mean, 15 days). On average, pregnancies were prolonged by 5.1 weeks (range, 2-8 weeks). The newborn weight was 2678 g on average (range, 620-3700 g). Oligohydramnios was seen in two instances; one patient developed constriction of the fetal ductus arteriosus, which returned to normality after indomethacin suppression; one newborn in which other causes of neonatal bleeding could be excluded, developed a disseminated intravascular coagulation and died 15 h after birth. Finally, one mother presented an acute renal failure immediately after indomethacin administration; this patient completely recovered after indomethacin withdrawal. Thus, the benefit of pregnancy prolongation should be balanced against the increased risks for the newborn, mainly fetal ductus arteriosus constriction and possible bleeding disorders. A causal relationship of indomethacin administration to the latter complication warrants further investigation.

Cardiac defects in chromosomally normal fetuses with increased nuchal translucency at 10-14 weeks of gestation.

OBJECTIVE: To examine the prevalence, distribution and spectrum of cardiac defects in chromosomally normal fetuses with increased nuchal translucency thickness. PATIENTS AND METHODS: During a 4-year period, targeted fetal echocardiography was used in 353 chromosomally normal fetuses with increased nuchal translucency thickness at 10-14 weeks' gestation. The cardiac scan was performed at 18-22 weeks. In the last 138 cases enrolled, an additional scan at 12-16 weeks was carried out. The follow-up included the findings at necropsy or in the pediatric examination. A complete follow-up was achieved in 97%. RESULTS: Cardiac defects were present in 32 (9.1%) cases, increasing from 5.3% in those with a nuchal translucency thickness of > or = 95th centile (3.9 mm) to 24% when thickness > or = 6 mm (p < 0.001). In 31 cases (97%), the cardiac defect was diagnosed antenatally; in 24 cases (77%) this diagnosis was confirmed later. In the remaining seven cases, the autopsy examination was not available. A wide range of cardiac defects was observed, with the most common being atrioventricular septal defect and tricuspid atresia. CONCLUSIONS: Euploid fetuses with increased nuchal translucency thickness have a significantly increased risk of cardiac defects. This is a marker of different types of heart anomalies and constitutes an additional indication for targeted fetal echocardiography. Most of the cardiac defects can be detected by fetal echocardiography.

[Percutaneous pulmonary valvulotomy using radiofrequency in pulmonary atresia with intact interventricular septum]. / Valvulotomía pulmonar percutánea mediante radiofrecuencia en la atresia pulmonar con septo interventricular íntegro.

Early pulmonary valvulotomy in patients with pulmonary atresia and intact interventricular septum allows the development and growth of the right ventricle and two-ventricle circulation. Percutaneous valvulotomy today is a valid alternative procedure to surgical valvulotomy. With the use of the radiofrequency 5F currently available for the treatment of arrythmias atretic pulmonary valve perforation and consecutive balloon dilation may be safely and effectively performed. We describe a case of perforation of a pulmonary valve by radiofrequency in a 15-day-old neonate with pulmonary valve atresia and intact ventricular septum.

[Transposition of the great arteries. Its anatomical correction. Our initial experience]. / Transposición de las grandes arterias. Corrección anatómica. Experiencia inicial.

Arterial switch correction of transposition of the great arteries has been performed without mortality on 8 children, 7 neonates and one 6 years old child, at our institution. One infant and the 6 year old child has previous pulmonary artery banding and modified Blalock-Taussig shunts. The coronary cuffs were anastomosed to the neo-aorta using the "trap-door" technique described by Mee and in seven the Lecompte manoeuvre was used. Postoperative management included treatment for persistent pulmonary hypertension and left ventricular dysfunction. All patients were discharged in satisfactory condition, and continue to do so at a maximum follow-up of ten months. These results encourage us to elect the arterial switch procedure as the primary treatment of transposition of the great arteries.

[Complete congenital atrioventricular block. Prenatal diagnosis and perinatal management]. / Bloqueo auriculoventricular completo congénito. Diagnóstico prenatal y manejo perinatal.

OBJECTIVE: To describe our experience in prenatal diagnosis and perinatal management of congenital atrioventricular heart block, as well as pacemaker treatment in the neonate. MATERIAL AND METHODS: A total of 13 fetuses are included. The diagnosis of atrioventricular dissociation was established by Doppler heart rate sample in the right atrium to show the atrial activity while the sample in the Aorta reflected the ventricular heart rate. Gestational age at diagnosis, ventricular heart rates, autoimmune maternal pathology, maternal blood tests for autoantibodies antiRo+, congenital structural heart disease, fetal hydrops, maternal medical treatment, perinatal results and pacemaker neonatal implantation are described. RESULTS: Gestational age at diagnosis ranged between 22 and 32 (mean 27.6) weeks. Ventricular heart rates ranged between 32 to 80 (mean 54) beats/min. AntiRo+ antibodies were detected in 5 mothers, and clinical systemic lupus erythematosus was found in only one. Four had congenital heart disease (2 ventricular inversion and corrected TGA, 1 complete atrio-ventricular canal and 1 tricuspid atresia). Signs of heart failure and hydrops were detected in 9 fetuses. Treatment with beta-metasona and ritodrine was administered to 7 mothers when the ventricular heart rate dropped below 60 beats/min. Intrauterine fetal death occurred in 3 fetuses with structural congenital heart disease and hydrops. Delivery was performed by cesarean section in 8 preterm fetuses (one them a twins), 3 spontaneous deliveries at term and 3 stillbirth. Postnatal pacemaker implantation was carried out in 9 newborns (3 cases with unicameral temporal right ventricle electrode and 6 cases with permanent bicameral electrodes implanted through the subclavian vein and DDD pacemaker). Follow-up of the bicameral pacemaker group was satisfactory. CONCLUSION: Persistent fetal bradycardia is the first sign to diagnose prenatal complete atrioventricular heart block. Echocardiography asses fetal haemodynamic status and may detect signs of fetal deterioration. Hydrops and further drop in the ventricular heart rate warrant urgent cesarean section and pacemaker management of the newborn.

[Neonatal intrapericardial teratoma]. / Teratoma intrapericárdico neonatal.

Neonatal intrapericardial teratoma is a rare cardiac tumor. The prognosis depends on making the diagnosis during the perinatal period. Surgery is usually resolutive with dramatic improvement of signs and symptoms of pericardial compression. We present a case of a female neonate diagnosed by prenatal echocardiography of intrapericardial teratoma at 34 weeks of gestation. Progressive increase of pericardial effusion and additional signs of atrial compression compelled to perform an elective Cesarean section at 38 weeks to be followed by an immediate surgical treatment. Pathology confirmed the diagnosis of mature teratoma. Postoperative course was favourable with radiologic and echocardiographic improvement.

Prenatal diagnosis of a right ventricular diverticulum complicated by pericardial effusion in the first trimester.

A ventricular diverticulum associated with a large pericardial effusion was diagnosed at 13 weeks of gestation. The pericardial effusion resolved spontaneously by 20 weeks and the diverticular size remained the same during pregnancy. In the postnatal period the neonate underwent surgical correction of both the diverticulum and associated ventricular and atrial septal defects. Our case indicates that congenital ventricular diverticulum may be associated with a good perinatal outcome.

Detection of persistent ductus in hypoplastic left heart syndrome by contrast echocardiography.

A patient with hypoplastic left heart syndrome was studied using contrast echocardiography after peripheral venous injection of dextrose. The combination of the parasternal and suprasternal approach allowed for identification of the right ventricular cavity, excluded the presence of right-to-left intracardiac shunts, and established normal ventriculoarterial connections. From the abdominal approach, the abdominal aorta was identified as an echo-free space corresponding to this structure in position, which became opacified after peripheral injections of dextrose into the left-hand vein. In view of the suprasternal and parasternal findings opacification of the abdominal aorta could only have occurred in the presence of a pulmonary artery to descending aorta shunt through a persistent ductus arteriosus.

Diagnosis of ventriculo-arterial discordance (transposition of the great arteries) by contrast echocardiography.

The constant anatomical relation of the aortic arch and the pulmonary artery bifurcation permits echocardiographic differentiation of the two great arteries when viewed from the suprasternal notch. When this technique was employed with contrast echocardiography in 12 infants, 6 of whom had ventriculo-arterial discordance (transposition of the great arteries), it was possible to establish with certainty in all but 1 case which great artery was most directly connected to the systemic venous return.

Echocardiography of primitive ventricle.

The angiocardiographic, echocardiographic, and, where available, the necropsy findings were correlated in 32 cases of primitive ventricle. Single probe echocardiography was shown to be a reliable and accurate technique for diagnosis of primitive ventricle; the ventricular and atrioventricular valve appearances were characteristic, and the outlet chamber was usually recognised when present, though it was not possible to say whether it was rigt or left sided. Abnormalities of the atrioventricular valves were more accurately shown by echocardiography than by angiocardiography though the two techniques were shown to be complementary in the overall diagnostic process.

Infradiaphragmatic total anomalous pulmonary venous connection to portal vein. Diagnostic implications of echocardiography.

A case of total anomalous pulmonary venous connection to the portal vein is described. The diagnosis was suspected clinically, supported by the echocardiogram, and confirmed by cardiac catheterisation, angiocardiography, and contrast echocardiography. An echo-free space lying behind the left atrium initially was thought to represent the common pulmonary vein. However, contrast echocardiography showed that this space was not the anomalous vein but probably an artefact. This paper shows that the origins of intracardiac echoes cannot always be assumed from a simple comparison of echocardiography with angiocardiographic or necropsy findings. In some cases it is necessary to introduce a marker into the echocardiogram which unequivocally originates from, and, therefore, localises, the structure under examination. Contrast echocardiography provides such a marker.

Routine prenatal ultrasound screening for fetal abnormalities: 22 years' experience.

We describe the results of routine obstetric ultrasound examination over a period of 22 years (1970-91) at the Institut Universitari Dexeus, Barcelona, Spain. A total of 1006 malformed fetuses or neonates were identified at abortion or delivery. The prevalence of fetal abnormalities was 3.03%. Fetal anomalies were diagnosed antenatally in 788 (78.33%) cases. Early prenatal diagnosis (before 22 weeks of gestation) was made in 598 (59.44%) cases. The detection of malformed fetuses increased from 19.75% in the first phase of the study (1970-74) to 96.33% in the last phase (1990-91). The specificity of the method was over 99% throughout the study period. The most frequently diagnosed anomalies were urinary tract anomalies (22.86% of cases), followed by head and neck anomalies (18.68%), musculoskeletal anomalies (8.64%), heart anomalies (7.35%) and gastrointestinal anomalies (7.35%). The earliest diagnosed malformations were those of the thoracoabdominal wall (81.08%), followed by defects of the urinary tract (70.86%) and of the diaphragm (70.83%).