[Complete vascular rings]. / Anillos vasculares completos.

The complete vascular rings, embryological anomalies of the aortic arch and great vessels, are frequently incidental findings, although patients with significant anomalies can present with upper airway and oesophagus compression, resulting in non-cardiac morbidity. The diagnostic needs high clinical suspicion. We describe six cases, with a mean age of two and a half months at diagnosis. The objective of our review is to describe the type of complete vascular ring (double aortic arch, right circumflex aortic arch and right aortic arch with aberrant left subclavian artery and left ligamentum arteriosus), the most common symptoms (respiratory symptoms including constant high-pitched, aggravated crying) and invasive and non-invasive diagnostic procedures (esophagograms, CT, MRI).

Anillos vasculares completos / Complete vascular rings

Los anillos vasculares completos, anomalías en el desarrollo embrionario del arco aórtico y grandes vasos, son frecuentemente hallazgos casuales; sin embargo, pacientes con anomalías importantes pueden presentar compresión de la vía respiratoria alta y esófago, con la consiguiente morbilidad no cardíaca que esta patología conlleva. Dado que para su diagnóstico se requiere un alto índice de sospecha clínica, el objetivo de la descripción de estos seis casos con una edad media en el momento del diagnóstico de 2,5 meses, es revisar el tipo de anillo vascular completo (doble arco aórtico, arco aórtico derecho circunflejo y arco aórtico derecho con arteria subclavia izquierda aberrante y ligamento arterioso izquierdo), las manifestaciones clínicas más frecuentes (síntomas respiratorios incluyendo el estridor constante, exacerbado con el llanto), y los métodos diagnósticos invasivos y no invasivos utilizados (esofagograma, tomografía computarizada, resonancia magnética) (AU)

The complete vascular rings, embryological anomalies of the aortic arch and great vessels, are frequently incidental findings, although patients with significant anomalies can present with upper airway and oesophagus compression, resulting in non-cardiac morbidity. The diagnostic needs high clinical suspicion. We describe six cases, with a mean age of two and a half months at diagnosis. The objective of our review is to describe the type of complete vascular ring (double aortic arch, right circumflex aortic arch and right aortic arch with aberrant left subclavian artery and left ligamentum arteriosus), the most common symptoms (respiratory symptoms including constant high-pitched, aggravated crying) and invasive and non-invasive diagnostic procedures (esophagograms, CT, MRI) (AU)

[Magnetic resonance imaging in long-term assessment after surgery for aortic coarctation]. / Valoración a largo plazo con resonancia magnética en pacientes corregidos de coartación de aorta.

OBJECTIVES: Spin echo or gradient echo magnetic resonance imaging provides enough information to plan patient management and can be completed with the use of three-dimensional magnetic resonance angiography to evaluate the need for reintervention, assess follow-up, or discharge the patient. PATIENTS AND METHODS: From 1990-2003, we evaluated 101 patients after initial corrective surgery for aortic coarctation at the age of 3.1 +/- 3 years, with subsequent postoperative assessment at 1 year and again 12.4 +/- 4.2 years later. No mortality was registered during the follow-up. Corrective surgery was performed in 32 patients (31.6 %) before the first month of life. All the patients were evaluated with spin-echo and echo gradient and 34 were evaluated with magnetic resonance angiography. The patients were classified into two groups: group A consisted of 68 patients (11 < 1 month old) with no complications. End-to-end anastomosis was performed in 55, the Alvarez technique in five, the Waldhausen technique in six, and conduit in two. Group B consisted of 33 patients complicated with early aortic recoarctation (21 corrected before the age of 1 month). End-to-end anastomosis was performed in 29, the Alvarez technique in two, and grafting in two. During the follow-up, 43 reinterventions were performed. Isthmus diameter/descending aorta diameter at the diaphragmatic level and repaired site diameter/descending aorta diameter at the diaphragmatic level were measured. RESULTS: Group A: the isthmus diameter/descending aorta diameter at the diaphragmatic level index was 0.92 +/- 0.08 and the repaired site diameter/ descending aorta diameter at diaphragmatic level index was 0.90 +/- 0.05. Twenty-three patients were assessed by means of three-dimensional magnetic resonance angiography, which revealed two late stenosis (one in a 10-year-old patient with an end-to-end anastomosis performed previously, and another in an 18-year-old patient with a proximal and distal obstructed conduit). Group B: the isthmus diameter/descending aorta diameter index was 0.84 +/- 0.1 and the repaired site diameter/ descending aorta diameter index was 0.82 +/- 0.11. Eleven patients were assessed by means of three-dimensional magnetic resonance angiography, which revealed five stenosis indexes (0.53-0.73) surgically corrected before the age of 2 months, four with an initial technique based on end-to-end anastomosis and one with a graft. CONCLUSIONS: Our results support the influence of young age, the use of end-to-end anastomosis and grafts in recoarctation and their late influence on recurrent recoarctation. The patients in group A were discharged in childhood or adulthood after periodic follow-up every 5 years with magnetic resonance angiography for 18 years with results within the normal range, while patients in group B required longer follow-up.

Tetralogía de Fallot con ausencia de rama pulmonar izquierda. Corrección quirúrgica / Tetralogy of Fallot with absence of the left pulmonary branch. Surgical correction

No disponible

[Tuberous sclerosis associated with rhabdomyoma in the left ventricular outlet tract]. / Esclerosis tuberosa con rabdomioma del tracto de salida del ventrículo izquierdo.

We report a 2-month old newborn with a family history of adenoma sebaceum, achromic spots and renal lithiasis. Echocardiography-magnetic resonance imaging showed a solitary pediculated rhabdomyoma in the left ventricular outlet tract causing a severe systolic aortic valve obstruction. Surgical excision was carried out through the aortic valve with no valvular lesions. The patient presented multiple seizures 24 hours after surgery with good response to medical therapy. Ten days later, computed tomography showed a spot lesion in the subependimary area in the posterior position of the parietal horn, compatible with a hamartoma associated with tuberous sclerosis.

[Criss-cross heart with atrioventricular discordance, straddling A-V valve, double outlet right ventricle and preexcitation]. / Criss-cross con discordancia auriculoventricular, acabalgamiento de la válvula auriculoventricular derecha, doble salida del ventrículo derecho y preexcitación.

We report the case of a newborn with cyanosis, dyspnea, and supraventricular tachycardia due to reentry with good response to medical therapy. The diagnosis was made by echocardiography. The patient died suddenly at 6 days of follow-up. Necropsy confirmed the following anomaly: criss-cross heart with straddling right atrioventricular valve, and left atrioventricular valve stenosis connected to the anterior trabeculated ventricle giving rise to the pulmonary artery and aorta.Precocious rotation when septal closure is not concluded favors atrioventricular valve anomalies and rhythm disorders.

[The treatment of postoperative junctional ectopic tachycardia]. / Tratamiento de la taquicardia ectópica de la unión tras la cirugía.

OBJECTIVE: To evaluate treatment of junctional ectopic tachycardia after cardiac surgery. MATERIAL AND METHODS: Twenty-seven patients (5.5 % of 488 patients who underwent surgery) were treated for junctional ectopic tachycardia between 1994 and 1998. There were 14 boys and 13 girls with a mean age of 11 11 months. Seven suffered from tetralogy of Fallot, seven from ventricular septal defect, six from atrioventricular septal defect, three from transposition of the great vessels and the remaining four had other complex heart diseases. The mean initial frequency was 186 27 beats/min. Crystalloid cardioplegia was applied in 274 patients (1994-1996) and 20 patients (7.4 %) showed junctional ectopic tachycardia. Hematic cardioplegia was performed in 214 patients (1997-1998) and seven patients (3.2 %) developed junctional ectopic tachycardia. Of the 33 patients who were treated during the surgical procedure with high mean doses of sympathomimetic catecholamine agents, 27 (81 %) developed tachycardia. Tachycardia developed 8.24 7 hours after surgery (range: 1-24 hours) in 25 patients and after 4 and 5 days in the remaining two patients. The mean duration of tachycardia was 4 days. RESULTS: In all patients rectal temperature was reduced to 32-34 C. Nineteen patients (70 %) showed a quick response (1-2 hours), although the technique was effective as an isolated procedure in only one patient. Sympathomimetic catecholamine level was reduced to 2-5 g/kg/min in 20 patients but this was effective in 14 (70 %). In 15 patients intravenous amiodarone was also administered and was effective in 11 patients (73 %). Finally, intravenous propafenone was administered to 5 patients. The most effective treatments were hypothermia with reduction of sympathomimetic catecholamine levels in 7 patients (100 %) or intravenous amiodarone in 4 (80 %). Tachycardia led to low cardiac output in 10 patients and only four recovered normal sinus rhythm. Eight patients died. Of these, hemorrhage in the junction area was confirmed in six patients. CONCLUSIONS: Junctional ectopic tachycardia is favored by high levels of sympathomimetic catecholamines after surgery. On the other hand, myocardial protection with hematic cardioplegia reduces tachycardia. Moderate hypothermia with reduction of sympathomimetic agents or intravenous amiodarone reverses ectopic tachycardia.

[Interventricular communication in the neonatal period]. / Comunicación interventricular en la edad neonatal.

OBJECTIVE: The aim of this study was to evaluate the prevalence and the follow-up in the long run of ventricular septal defect in the neonatal period. MATERIAL AND METHODS: 222 patients with ventricular septal defect from a total amount of 30,840 newborns admitted to our Hospital were evaluated between January 1991 and December 1996 (The incidence accounts for 7.19 per 1,000 newborns). 123 of these patients were males (55.4%) and 99 females (44.6%). The mean age at the time of examination was 3.09 +/- 1.67 days (range from 1 to 11 days). Diagnosis was suspected because of a harsh systolic murmur in 207 cases (93.2%), a malformative syndrome in 10 (4.5%) or polypnea in 5 (2.3%). The study was performed with a Sonos 1000 Echocardiograph device. The mean follow-up period ranged from 1 to 72 months. 10 patients underwent cardiac catheterization (4.5%). RESULTS: A muscular defect was the common finding in 177 patients (79.7%) whereas 41 patients (18.5%) exhibited a perimembranous defect. The mean size defect was 2.9 +/- 1.2 mm (range 1-10 mm), being smaller in the muscular defects (2.56 +/- 0.68) than in the perimembranous defects (4.33 +/- 1.56) p < 0.001. Spontaneous closure occurred in 176 ventricular septal defects (79.3%), 155 of them corresponding to muscular defects (87.6%) and 18 to perimembranous defects (43.9%). The mean age at the time of spontaneous closure was 9.5 +/- 8.9 months (9.4 +/- 9 months in the muscular defects and 10.3 +/- 4 months in the perimembranous defects). Spontaneous closure occurred by age 6 months in 52% of the patients with ventricular septal defect (54.5% muscular defects, 33.3% perimembranous defects), 12 months in 72.7% (71.2% muscular defects and 83.3% perimembranous defects) and 24 months in 96% (96.1% muscular defects and 94.4% perimembranous defects). 10 patients underwent surgical repair by age 11 +/- 8 months. All of them exhibited perimembranous defects with a mean size 6 +/- 1.5 mm which were tantamount to 24.4% of this group. 3 patients died (1.4%), 2 suffering from trisomy 18 and 1 after cardiac surgical repair. CONCLUSION: The most common ventricular septal defects in the neonatal period occur in the region of the muscular septum. The prognosis is favourable. Most of the patients experience spontaneous closure and show a very low mortality rate which is basically related to extracardiac anomalies.

[Magnetic resonance of congenital cardiopathies]. / Resonancia magnética en las cardiopatías congénitas.

OBJECTIVE: The objective of this study was to determine the usefulness of magnetic resonance in the diagnosis and follow-up in congenital heart disease. PATIENTS AND METHODS: Between 1992 and 1994, we have evaluated 250 patients (between 1 day-22 years of age) with congenital heart disease. We used a Philips-Gyroscan 0.5 Tesla set-up and the studies were made with ECG-gated multislice sequences with the time of repetition adapted to the cardiac frequency. The slice thicknesses were 3-10 mm and the field of view was 100-400 mm. Three axes were used: axial, oblique coronal and 30-60 degrees oblique sagittal. Three groups were made: A) Patients in whom MR imaging was the elected technique for establishment of the diagnosis; B) Patients were MR imaging was an alternative technique; and C) Patients were MR imaging was complementary to other techniques. All patients underwent a Doppler echocardiographic study or an angiocardiogram. RESULTS: Group A) One hundred and fifty-one patients were included with the following conditions: Eighty-two with coarctation of the aorta or aortic arch interruption, 12 with vascular ring, 5 with truncus arteriosus in order to evaluate the pulmonary branches, 28 cases operated for Fallot or transposition of the great vessels for the evaluation of the pulmonary branches, 16 with single ventricle for the evaluation of the bulboventricular foramen, 5 with complex congenital heart disease and 3 with pericardial agenesia-herniation. Group B) Sixty-five patients were included in this group with the following diagnoses: Ten with aorto-pulmonary colateral arteries, 13 with anomalous pulmonary drainage of the systemic veins, 38 with tronco-conal anomalies and 4 with supravalvular aortic stenosis. Group C) Thirty-four patients with the following conditions were included in this group: Ten with common atrioventricular septal defects, 6 with tricuspid atresia, 6 with atrioventricular and ventriculoarterial discordance and 12 with other anomalies. CONCLUSIONS: MR imaging can replace other techniques in the diagnosis and follow-up of some congenital heart diseases and in a non-invasive way.

[Down's syndrome in the Basque autonomous community, 1990-1995: types of birth and follow-up of a cohort of 116 children during the first year of life. Registry of anomalies]. / Síndrome de Down en la Comunidad Autónoma del País Vasco, 1990-1995: Tipos de nacimiento y seguimiento de una cohorte de 116 niños durante el primer año de vida. Registro de anomalías congénitas de la Comunidad Autónoma del País Vasco (RACAV).

OBJECTIVE: The aim of this study was to assess the frequency of associated anomalies in babies with Down's syndrome (DS) and their progress throughout the first year of life. PATIENTS AND METHODS: All pregnancies of women resident in the Basque Autonomous Community with prenatal or postnatal diagnosis of DS ascertained during the period 1990-1995 were studied. A one year follow-up of all DS babies was carried out. RESULTS: One hundred and sixteen live born DS babies were identified. Half of them (58) showed other associated anomalies. Congenital heart disease (CHD) was the most frequent anomaly, present in 45 cases. Atrioventricular canal (AVC) was the most frequent type of CHD, present in 34 out of the 45, the complete from being more prevalent (2/3). A marked association between AVC and female sex was observed. The overall mortality in the first year of life was 18.1%, associated to the presence of CHD. CONCLUSIONS: These findings are in keeping with the observations reported in previous similar studies. Consistency of these population-based data over time provides and accurate and reliable basis both for planning the needs of children with DS and to counsel affected parents.

[Wolff-Parkinson-White disease in childhood: follow up of 36 cases]. / Wolff-Parkinson-White en la infancia: seguimiento de 36 casos.

We have studied 36 patients (19 females and 17 males), controlled between 1973 and 1989, who suffered Wolff-Parkinson-White (WPW) pattern in their electrocardiogram. Epidemiological, clinical, diagnostic, therapeutic and evolutional data were reviewed. The mean age at the time of diagnosis was 4 years and 3 months, with 48% younger than six months of age. The average time for the follow-up period was 4 years and 2 months. There was not familiar occurrence. Six (16%) of the patients had associated heart disease. Seventeen (47%) had type A of WPW, 12 (33%) type B and 7 (20%) were not defined. An echocardiographic study was done in 20 patients (61%). Twenty-seven patients (75%) showed supraventricular tachycardia (SVT), which in 22 of these cases was the reason for seeking consultation. Children without SVT, 9 (25%), did not need any form of treatment. Twenty-four (889) of the patients with SVT required treatment to prevent recurrence. In the 27 studied episodes of SVT, Verapamil IV (55%) and vagal manoeuvres were the most efficient treatments. Seventeen (47%) of the patients presented a persistent WPW pattern and 11 (31%) experienced a normalization of their electrocardiogram with a mean time of 2 years-2 months.

[Doppler echocardiography in right and left obstructive cardiopathies]. / El Doppler en las cardiopatías con obstrucción derecha e izquierda.

In order to evaluate the usefulness of Doppler echocardiography (continuous wave) in the assessment of obstructive cardiopathies, we studied the patients with pulmonary or aortic valvar stenosis examined from January 1988 to August 1989 in our Hospital. Doppler echocardiographic studies were performed on 35 patients, 19 with pulmonary valvar stenosis and 16 children with aortic valvar stenosis. Pulmonary flow was recorded from the left parasternal position (3-4 intercostal space) and aortic flow from the right parasternal position (3-4 intercostal space), apical and suprasternal notch window. We calculate the pressure gradient by the modified Bernoulli equation. The mean right Doppler gradient was 62 +/- 14 mmHg (ranged from 45 to 95) and the mean left Doppler gradient 64 +/- 29 mmHg (ranged from 30 to 150). The accuracy of Continuous Wave Doppler Echocardiography technique in our patients was verified by linear regression analysis of doppler-calculated and catheterization-measured gradients when the procedures were performed sumultaneously or within 48 hours. The statistical analysis included 19 children with pulmonary valvar stenosis and 16 with aortic valvar stenosis. This study confirms that Doppler echocardiography technique is an accurate and simple method to assess the cardiopathies with outflow obstruction. We believe that catheterization-measured gradients can be replaced by doppler-calculated ones, since there is an excellent correlation between them when they are performed simultaneously.

[Congenital absence of the pulmonary valve. Analysis of 7 cases]. / Ausencia congénita de la válvula pulmonar. Estudio de siete casos.

We present seven cases of congenital absence of pulmonary valve observed during a 15 years period. There are five females and two males, aged from one day to six years. Three patients with a birth weight under 1.500 g died in the first 24 hours. All the patients had a systolic diastolic murmur and a single second sound. Two dimensional echocardiography was the procedure that allowed a definite diagnosis. In the valvular ring area a fixed ecogenic image was observed during the cardiac cycle. The pulmonary trunk and branches were dilated and the valvular ring was normal or small. In four patients the study was completed with catheterism and angiocardiography. Two were operated upon, with closing of the ventricular septal defect, aortic hemograft in pulmonary situation and reduction of pulmonary artery branches size.

[Value of the esophagogram in the study of vascular rings]. / Valor del esofagograma en el estudio de los anillos vasculares.

The diagnostic value of esophagograms was studied in 67 patients with aortic arch abnormalities and tracheoesophageal compression. This group of patients included the following malformations: double aortic arch (11), retroesophageal subclavia (48), retroesophageal aorta (5), cervical arch with retroesophageal component (2) and anomalous left pulmonary artery (sling) (1). Three types of esophageal indentation were observed in the esophagogram: anterior, posterior and bilateral. Anterior indentation was caused by the left pulmonary artery, and posterior indentation, when isolated, was caused by the retroesophageal subclavia. Posterior indentation associated with bilateral indentation was present in cases of double aortic arch. The upper lateral indentation was always caused by the predominant arch. We conclude that esophagogram is an easy and valid method for the diagnosis of this type of pathology.

[Restricted intake of sodium in newborn infants with very low birth weight in the first days of life]. / Aporte restringido de sodio en el recién nacido de muy bajo peso en los primeros días de vida.

A prospective study on the amount of sodium administered in the first five days of life in a group of 18 low-birth-weight infants is presented. Their mean birth weight, and gestational age were 1.150 gm., and 28.8 weeks. Patients were cared for in radiant warmers, and protected with a plastic blanket. Minimum amount of sodium necessary to maintain a normal plasma sodium concentration was given. Mean daily sodium and free water given was 0.96 mEq/kg/day, and 108.4 ml/kg/day, respectively. In day 5, there was a mean accumulated body weight loss of ten per cent. This group of patients were compared with a similar group in whom plastic blanket was not used, and who received a sodium intake of 1.5 mEq/kg/day. In this latter group of patients there was a higher incidence of hypernatremia (p less than 0.005). The incidence of hyponatremia was similar in both groups. Daily sodium intake might be very low, in infants of very-low-birth-weight if a negative weight balance is allowed in the first days of life.