Cardiopatías congénitas. Un problema pediátrico que no acaba en la edad pediátrica / Congenital heart diseases. An unfinished pediatric problem in the pediatric age

Las cardiopatías congénitas constitutyen la malformación congénita más frecuente. Los avances médicos y quirúrgicos realizados en la edad pediátrica han hecho posible una considerable disminución en la mortalidad, por lo que el 85% de estos pacientes llegan a la edad adulta, pero lo hacen con un corazón reparado y en ocasiones con un patrón circulatorio diferente al normal, constituyendo una nueva y compleja patología hasta hace poco inexistente. Para la atenicón de estos pacienes es preciso configurar unos modelos de atención y una red aistencial todavia en gestación. En el artículo se explica cómo uno de los hospitales reconocidos por su consolidada trayectoria en la atención de esta patología durante la edad pediátrica ha resuelto este problema, crando un programa transversal de Cardioloía d elas Cardiopatías Congénitas que se ocupa de esta patología a lo largo de todo el continuum vital del paciente (AU)

Congenital heart defects are teh most common congenital malformation. The medical and surgical advances made in pediatric patients have made possible a considerable reduction in mortality, so that 85% of patients reach adulthood, but do so with a heat repaired and sometimes with a different circulation pattern than normal, forming a new and complex pathology until recently nonexistent. For the management of these patients must be configured models of care and assistance netword. The article expalins how one of the hospitals recognized by its established track record in dealing with this disease in childhood has solved this problem by creating a transversal program of Congenital Cardiology for dealing with thid disease along entire continuum of patient life (AU)

Myocardial perfusion magnetic resonance imaging for detecting coronary function anomalies in asymptomatic paediatric patients with a previous arterial switch operation for the transposition of great arteries.

BACKGROUND: The main cause of long-term morbidity and mortality after the arterial switch operation for transposition of great arteries is complication at the coronary arteries. Myocardial perfusion magnetic resonance imaging represents a relatively novel and appealing tool for detecting myocardial ischaemia but with little experience in paediatric patients. The purpose of this paper is to report a single centre experience with myocardial perfusion magnetic resonance imaging for detecting ischaemia after the arterial switch operation for transposition of great arteries. METHODS: Twenty-eight patients aged 13-16 years with an arterial switch operation for transposition of great arteries were included in the study. Coronary pattern, operative and postoperative complications, and long-term follow-up events were reviewed. Patient functional evaluation included clinical examination, electrocardiogram and echocardiogram. Every patient underwent magnetic resonance imaging perfusion scanning at rest and under adenosine-induced stress. RESULTS: All patients were symptom free with no ischaemic signs on the electrocardiogram. All magnetic resonance imaging examinations were generally well tolerated with minor adenosine secondary effects in 36% of the patients. Two stress myocardial perfusion magnetic resonance studies were excluded from analysis for technical reasons. No perfusion stress defects were detected at the remaining 26. Myocardial delayed enhancement was performed in all 28 patients. In five subjects, a subendocardial late enhancement consistent with patch tissue for septal defect closure at the time of repair was indentified. CONCLUSION: Magnetic resonance imaging evaluation of myocardial perfusion and viability is feasible in paediatric patients long after arterial switch operation. No signs of myocardial ischaemia or necrosis were documented in this young asymptomatic population. Further studies including coronary angiography correlation are needed to validate magnetic resonance imaging results.

Late outcome of Senning and Mustard procedures for correction of transposition of the great arteries.

OBJECTIVES: To evaluate late mortality and morbidity after an atrial switch procedure for correction of transposition of the great arteries (TGA) and to assess predictive factors for adverse outcome. SETTING: Tertiary referral centre. DESIGN AND PATIENTS: Retrospective follow up study of 137 patients surviving hospitalisation for TGA atrial switch procedure (Mustard or Senning) in a single institution and divided into two groups (simple and complex) depending on presurgical anatomy. Several surgical and follow up factors were evaluated during 16.7 (5.6) years' follow up. RESULTS: Late mortality was 5.1% (95% confidence interval 1.37% to 8.84%) with sudden death as the most common cause. No significant difference was found between Mustard and Senning procedures and between the complex and simple groups in terms of mortality. Independent predictive factors for late mortality were a history of supraventricular tachyarrhythmias and advanced New York Heart Association (NYHA) functional class during follow up. A very common finding was development of sinus node dysfunction (47.6%), which had no influence on mortality. There was little need for reintervention (5.1%) and relatively few cases of right ventricular systolic dysfunction (14.6%). During follow up, most patients (96.2%) were in NYHA functional class I-II. CONCLUSIONS: Overall long term outcomes of patients with atrial repair of TGA in the present era are encouraging in terms of late mortality and quality of life. Nevertheless, better outcomes may be offered through improved diagnostic methods for right ventricular function and better management of supraventricular tachyarrhythmias.

[Total anomalous pulmonary venous return in children: importance of echocardiographic diagnosis and early surgery]. / Retorno venoso pulmonar total anómalo en pediatría: importancia del diagnóstico ecocardiográfico y de la cirugía precoz.

OBJECTIVE: We studied patients who underwent surgical repair for total anomalous pulmonary venous return at our hospital. We report the importance of diagnosis by echocardiographic imaging before surgical treatment. METHODS: Within the period of 1990-1999, fourteen patients underwent surgical repair of this cardiopathy in our hospital. The type of anomalous drainage was supracardiac in 6 patients, infracardiac in 4, to the coronary sinus in 1, and mixed-type in 3 patients. Eleven cases were diagnosed with an echo-Doppler study, the findings being confirmed intraoperatively. RESULTS: There were 2 early deaths: one occurred in the operating room in a patient with a small left ventricle, and the second one was 35 days postoperatively as a result of a septic complication. Early in the postoperative period our primary goal has steadily been the control and treatment of pulmonary hypertension. After a mean follow-up time of 50 months, only 1 patient needed to be reoperated on and the remainder are symptomless. CONCLUSIONS: That sufficient diagnostic data on total anomalous pulmonary venous return can reliably be obtained by ultrasound scanning so that surgery can be promptly undertaken, and that its surgical risk is currently low and mid-term post-repair outcome is fairly good.

Heparin therapy and reversal of protein-losing enteropathy in a case with congenital heart disease.

We describe a patient with tricuspid hypoplasia who developed a protein-losing enteropathy. Having failed to respond to medical and surgical treatments, a heparin regimen was started with immediate decrease in enteric protein loss.

[Arterial switch: aortocoronary bypass with interposition of polytetrafluoroethylene (Gore-tex) vascular graft]. / Switch arterial: bypass aortocoronario con interposición de injerto vascular de politetrafluoroetileno (Gore-tex).

We report a case of an infant with transposition of the great arteries accompanied by an unusual coronary artery pattern, in whom an expanded polytetrafluorethylene (Gore-tex) graft to the right coronary artery was used during surgical correction (switch arterial), with a postsurgical follow-up time of 8 months.

[Familial auricular fibrillation]. / Fibrilación auricular familiar.

INTRODUCTION AND OBJECTIVE: We present two families with atrial fibrillation in 20 of 50 members, during three generations, with known cardiac rhythms, in order to communicate their infrequent existence and the most relevant clinical facts. METHOD: Clinical situation, evolution, ECG and ECHO findings, treatments and complications related with the disease are investigated. RESULTS: The presence of atrial fibrillation in 20 members is demonstrated, although one of them was on sinus rhythm at the time of the study; 3 patients had left ventricular enlargement on the ECHO study; the clinical situation was good in all patients except two who died because of complications related to the arrythmia and a third patient that had a brain stroke. The patients received different treatments because they where controlled by different physicians; the possible lethal proarrythmic effect in such cases must be taken into account. CONCLUSION: Familiar atrial fibrillation is a very infrequent arrythmia, usually well tolerated, that follows a dominant autosomic hereditary pattern. The use of antiagregants is advised because of the risk of embolism, or the use of anticoagulants in the presence of associated risk factors. Electric cardioversion has been show not be useful. The possible proarrythmic effect of some antiarrythmic agents, used in the control of cardiac frequency, must be taken into account.

[Double-outlet left ventricle with subaortic interventricular defect and pulmonary stenosis: surgical correction with cryopreserved aortic homograft]. / Doble salida ventricular izquierda con comunicación interventricular subaórtica y estenosis pulmonar: corrección quirúrgica con utilización de homoinjerto aórtico crioconservado.

Double outlet left ventricle with subaortic ventricular septal defect and pulmonary stenosis is a very rare congenital heart disease. We present a case of this cardiopathy which underwent correction consisting of ventricular septal defect closure and right ventricular outflow tract reconstruction using a cryopreserved aortic homograft, with a postsurgical follow-up time of 18 months.

[Doppler echocardiography in the postoperative assessment of the arterial switch in the repair of transposition of the great arteries and double outlet right ventricle]. / Ecocardiografía Doppler en la valoración posquirúrgica del switch arterial para la reparación de la transposición de las grandes arterias y la doble salida del ventrículo derecho.

OBJECTIVE: To evaluate the usefulness of Doppler-echocardiography in the follow-up of anatomical repair in transposition of great arteries and double outlet of right ventricle. PATIENTS AND METHODS: 108 Doppler-echo studies, in a follow-up protocol of the first 32 consecutive patients who successfully passed anatomical repair, were analyzed. The mean age for operation was 50 days (6-472 days) (25 patients before 21 days of age) with a mean follow-up time of 406 days (1 day-4.5 years). We studied subjective impression of left ventricular function and its quantification by systolic shortening fraction, the functioning of four heart valves; diagnosis and quantification of stenosis in arterial sutures of neoaorta and neopulmonary, the most useful windows and planes and, finally, the difficulties found during the examination. RESULTS: Thirteen patients (41%) showed a low left ventricular shortening fraction, though 85% of these patients had anomalous ventricular septal motion. Neoaortic valve insufficiency was detected in 12 patients (37%) (mild in 10 and moderate in 2) and neopulmonary valve regurgitation in 14 patients (44%) (mild in 11 and moderate in 3). Stenosis of arterial sutures was detected in: 2 (6%) patients at neopulmonary artery and 1 (3%) at neoaortic artery. The biggest technical difficulty was found in the neopulmonary artery evaluation. CONCLUSIONS: 1) Doppler echocardiography is an excellent method for anatomical and functional evaluation in these patients and is necessary in their follow-up evaluations; 2) Surgical results obtained in this series are good; 3) Left ventricle fractional shortening is misleading in the evaluation of ventricular function in these patients because of the high incidence of abnormal ventricular septal motion, and 4) The study of neopulmonary arteries is often very difficult.

[Left aorto-ventricular tunnel. Diagnosis with transthoracic and transesophageal echo-Doppler]. / Túnel aorto-ventricular izquierdo. Diagnóstico mediante eco-Doppler transtorácica y transesofágica.

Aortic-left ventricular tunnel is an infrequent congenital heart lesion consisting of an abnormal tubular-shaped communication between the aortic root and the left ventricle that bypasses the aortic valve. It is clinically undistinguishable from aortic insufficiency. Its prompt and precise diagnosis is crucial since early surgical treatment is definitely indicated, in contrast to aortic insufficiency. In our case, two-dimensional and pulsed color-flow Doppler imaging established the diagnosis, and the transesophageal approach was a great aid in detecting the aortic opening with its turbulent diastolic flow, and in tracing its intramyocardial course. This paper may be of interest not just as a new case-report but insofar as it stresses how importantly the transthoracic and transesophageal echocardiographic and Doppler scanning contributes to its diagnosis and therapeutic indication.

[The follow-up of transposition of the great arteries corrected by Senning's technic]. / Seguimiento de la transposición de grandes arterias corregida con técnica de Senning.

INTRODUCTION AND OBJECTIVES: The incidence of late complications after a physiological correction of the patients with transposition of the great arteries (D-TGA) is very significant, due to the alternative operation of the arterial switch. METHODS: We studied 125 patients with D-TGA, treated with Senning surgical correction between december of 1978 and november of 1990. Surgery was performed at a mean age of 11.7 months (from 7 days to 11.2 years), and the postoperative mean follow-up was 7.3 years (from 1.4 to 14.3 years). We analyzed their evolutive clinical condition, ECG, Holter and echocardiogram-Doppler. Four groups were defined: A) Simple, 48.8%. B) Associated with ventricular septal defect, 22.4%. C) With pulmonary stenosis, 15.2%. D) Both anomalies, 13.6%. RESULTS: Sixteen children died (12.8%), 11 of them on the postoperative period. The remaining 5 patients died, at a mean time of 34.3 months after surgery, because they were in cardiac failure. All of patients had enlargement of right ventricle and tricuspid regurgitation was observed in 39 children. There were 3 reoperations. Atrioventricular block was observed in 5.7% of the patients, 33.3% were not in sinus rhythm, 6.6% had atrial flutter-fibrillation, sinus node dysfunction was observed in 24.7%, and five permanent pacemakers were implanted (4.7%). CONCLUSIONS: The later mortality is not high, and the clinical outcome is good, but the frequent rhythm disturbances and enlargement of the right ventricle could let us conclude the hypothesis that anatomical correction is an optimal alternative procedure.

[Evaluation of the efficacy of chelation therapy with deferoxamine in patients with thalassemia major]. / Valoración de la eficacia del tratamiento quelante con desferrioxamina en pacientes afectados de talasemia maior.

BACKGROUND: The current treatment of thalassaemia maior (TM) is based on a hypertransfusion regimen, with deferoxamine (DFO) chelation therapy to minimize the consequences of iron overload. To evaluate the long-term efficacy of chelation therapy, a group of 9 patients treated for a period of 9 years was studied. METHODS: The mean age of patients at the beginning of chelation therapy was 7 years. The age range at the moment of the study was 11 to 21 years. Pre-transfusion haemoglobin values were maintained above 10 gr/dl. DFO was administered by 10-hour sub-cutaneous infusion, 5 or 6 days a week at a dose of 40 mg/kg. Different iron overload parameters were considered, with special attention to cardiac function, growth and endocrinologic development. Signs of DFO toxicity were also studied. RESULTS: The final mean iron elimination rate was 72.6%. One patient died from cardiac haemosiderosis. Eight of the 9 patients showed significant growth impairment and 7, who have attained puberal or post-puberal age, suffer from one or more endocrinologic disorders (6 hypogonadism, 2 diabetes mellitus, 2 hypothyroidism and 1 hypoparathyroidism). The only toxic effect observed was transient crystalline opacity in 2 patients. CONCLUSIONS: Despite the early initiation of chelation therapy, TM patients receiving hypertransfusion regimen showed iron overload, with myocardiopathy, growth retardation and several endocrinologic disorders, mainly secondary hypogonadism, glucose metabolism disfunction and primary hypothyroidism.

[Anatomical correction in transposition of the great arteries and double-outlet right ventricle. Our initial experience]. / Corrección anatómica en la transposición de grandes arterias y en la doble salida de ventrículo derecho. Experiencia inicial.

INTRODUCTION AND OBJECTIVES: Anatomical correction of transposition of great arteries and double outlet right ventricle with subpulmonary ventricular septal defect is a surgical approach that has not been generally adopted in our clinical environment. Our aim with this paper is reporting on our initial experience with this technique. METHODS: The clinical data and additional investigations are reviewed from 15 infants with transposition of the great arteries and 2 with double outlet right ventricle who underwent anatomical repair, with a postoperative follow-up of one year. RESULTS: The survival rate has been 76% (13 out of 17 cases). The 13 survivors are in good hemodynamic condition, with no treatment whatsoever. Thirteen patients are in sinus rhythm with normal repolarization patterns and 5 patients show a right bundle branch block. Neither aortic nor pulmonary gradients have been detected on Doppler examination, and slight valvular insufficiencies are found at aortic level in 4 patients, pulmonary in 2 and mitral in one. CONCLUSION: Anatomical correction is the method of choice for transposition of the great arteries and double outlet right ventricle with pulmonary-related ventricular septal defect.

[Anomalous origin of the right pulmonary artery from the ascending aorta. Its echo-Doppler diagnosis]. / Origen anómalo de la arteria pulmonar derecha desde la aorta ascendente. Diagnóstico eco-Doppler.

The anomalous origin of the right pulmonary artery from the ascending aorta is a rare malformation. Its diagnosis and early surgical treatment are necessary to avoid its fatal outcome. We present the pulsed and color echo-Doppler findings in a patient with this pathology.

Unsuspected rheumatic heart underlying group B streptococcal endocarditis at the age of 20 months.

The case is reported of a 20-month-old girl admitted to our centre for group B streptococcal endocarditis who died of complete atrioventricular block after a week of treatment. There was no history pointing to the presence of a heart disorder. Necropsy disclosed the surprising existence of a vast rheumatic carditis involving essentially the aortic and mitral valves, with bacterial superinfection of the former. The double rheumatic and infectious lesion in such a young patient with infective pseudo-aneurysms of the sinuses of Valsalva makes this an exceptional case.

Hypertrophic cardiomyopathy in mucopolysaccharidoses: regression after bone marrow transplantation.

Mucopolysaccharide storage disease (MPS) presents clinically with a broad spectrum of abnormalities, among which cardiovascular involvement has been described. The echocardiographic findings have recently been reported for the various types of MPS. Among these, asymmetric septal hypertrophy (ASH) has been documented. We present a case of a 9-year-old girl suffering from type I MPS, atypical variant, with echocardiographic signs of ASH. She was given a bone marrow transplant after which the hypertrophic cardiomyopathy regressed.

[Results of valvuloplasties with balloon catheter in children. Follow-up using pulsed Doppler. Experience with our first cases]. / Evolución de las valvuloplastias con catéter balón en la edad pediátrica. Seguimiento mediante Doppler pulsado. Experiencia en nuestros primeros casos.

Our series includes the first 19 consecutive patients with pulmonary valvar stenosis who underwent balloon valvuloplasty in our hospital. All of them have been evaluated by an echo-Doppler study at different follow-up times, and in 10 patients the study was also performed prior to the dilatation. The ages ranged between 0.4 and 10 years (mean 3.5 years; standard deviation [SD] 2.53 years). The prevalvuloplasty degree of pulmonic stenosis found at catheterization was quantified as severe in 8 cases (42.11%) with a right ventricular systolic pressure (RVSP) in excess of 90 mmHg, and moderate in 11 cases (57.89%) with an RVSP from 70 to 90 mmHg. Transvalvular gradients were registered between 38 and 110 mmHg (m: 69 mmHg and SD: 15 mmHg). According to catheterization data obtained at the time of valvuloplasty, the procedure was considered ineffective in 2 cases (10.52%), scarcely effective in four (21.05%), and successful in the remaining 13 cases (68.42%). Doppler follow-up was carried out between 1.6 and 31.9 months after valvuloplasty (mean 12.9 months and SD: 10 months). Residual stenoses were estimated by Doppler as mild in 13 cases (68.42%), moderate in four (21.05%) and severe in two (10.52%). Mild pulmonary regurgitation was detected in 10 patients (52%). The 13 patients with a satisfactory result had slight residual gradients at the valvular level that had not been modified with the course of time. Of the 4 cases with a poorly effective dilatation the gradients noted by Doppler increased in three of them, being valvular in all but one in whom the obstruction was catalogued as infundibular.(ABSTRACT TRUNCATED AT 250 WORDS)