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A case report on the perinatal management of a 30-week preterm baby with congenital complete heart block
Abstract
Congenital complete heart block is an uncommon condition in the newborn, but is known to occur with maternal systemic lupus erythematosus.
This paper presents one such baby with complete heart block who was born premature (after a gestation of 30 weeks) and weighing 759 g.
Continuous isoprnaline infusion was initially used to support the baby while her other neonatal problems were treated. A Medtronics VV1 pacemaker was subsequently inserted to maintain a heart rate that would be more physiologically acceptable for the patient.
This baby is currently thriving well, having been followed up for one year.
The management issues, encompassing maternal and neonatal problems, and a review of current literature on this condition are discussed.
... Hay reportes en la literatura de neonatos menores de 1.500 gramos, incluso uno de 30 semanas y 759 gramos, con resultados satisfactorios a un año de seguimiento. Se espera que con la detección de más casos de esta patología no muy frecuente, más la experiencia y la llegada de dispositivos de tamaño óptimo, pueda brindársele una excelente atención a estos pacientes y así velar por su mejor pronóstico y sobrevida a largo plazo (18,19,20,21) . ...
Se presenta el caso de un recién nacido con diagnóstico de Lupus Neonatal del Servicio de Neonatología del Hospital Nacional Dos de Mayo en Lima, el cual se sospechó por tener bradicardia severa y bloqueo aurículo ventricular de IIIo. Durante la etapa prenatal presentó bradicardia fetal con frecuencia cardiaca promedio de 80 latidos por minuto y test de bienestar fetal normal. La madre no presentó antecedentes patológicos. Nació por cesárea por sospecha de sufrimiento fetal o cardiopatía congénita, con líquido amniótico claro y APGAR 81 – 85. Cursa con frecuencia cardiaca que llegan a 60 latidos por minuto. El EKG evidenció bloqueo aurículo ventricular de IIIo, se realizó la investigación de las causas de bloqueo cardiaco de este tipo y se tuvo resultados positivos para IgG anti SSA/Ro. Al mes de vida se le colocó marcapasos cardiaco.
... En nuestro medio no se encuentran datos al respecto.En cuanto a la literatura mundial, existen diversos reportes de implantación de marcapasos definitivo en recién nacidos a término y prematuros a nivel abdominal y retroperitoneal, con tasas de éxito y sobrevida muy aceptables(27)(28). Existen reportes de neonatos menores de 1.500 gramos(30,31,32), incluso de 30 semanas y 759 gramos, con resultados satisfactorios a un año de seguimiento(33). Se espera que con la detección de más casos de una patología no muy frecuente, más la experiencia y la llegada de dispositivos de tamaño óptimo, pueda brindársele una excelente atención a estos pacientes y así velar por su mejor pronóstico y sobrevida a largo plazo. ...
Complete congenital atrioventricular block is a rare entity that has a high morbidity and mortality. Its real incidence remains unknown and a high suspicion index is needed for its diagnosis and consequently for its early intervention. It is observed in children of mothers having connective tissue autoimmune diseases, in particular systemic lupus erythematosus, when the condition is congenital. If it is post-natal, congenital cardiopathies are responsible in most cases. It may also appear in structurally normal hearts. The characteristic clinical finding is persistent bradycardia manifested since intrauterine life and affecting the circulatory fetal stability, going as far as to produce hydrops fetalis, a serious and lethal condition. After birth, it appears with bradycardia as well, that may or not unbalance the patient hemodynamics. Diagnosis is made upon clinical suspicion with fetal echocardiography and when post-natal, through electrocardiogram and maternal antibody type antiRo and antiLa. Pacemaker implantation is the definitive treatment that contributes to improve patient survival and prognosis.
Isoproterenol (ISO) administration produces significant biochemical and histological changes including oxidative stress, reactive oxygen species (ROS) overproduction, and inflammation that leads to aggravation of myocardial injury. Subcutaneous or intraperitoneal ISO injection into rats can replicate several features of human heart disease, making it a useful tool for comprehending the underlying mechanisms and evaluating potential therapeutic strategies. In the present chapter, we elaborate on how depending on the precise experimental goals and the intended level of severity, different dosages and regimens are employed to induce myocardial injury.
This chapter describes important features of the fetal circulation, essential changes at birth and other age-specific developments of the cardiovascular system. The incidence, prevalence and basic pathophysiological principles of congenital heart defects are presented. Several special situations are discussed in more detail: the exercise physiology of patients with repaired congenital heart disease, the Fontan physiology and the transplanted heart. Indications and practical considerations for the use of various vasoactive and antiarrhythmic drugs are reviewed.
Advances in fetal echocardiography are providing highly accurate diagnoses of congenital heart disease prior to delivery, making it possible to plan the delivery-room management of these newborns. Knowledge of the expected transitional circulation occurring with birth and the pathophysiologic implications of congenital heart disease increases the likelihood of providing efficient and effective therapies. The majority of neonates who have congenital heart disease will not require delivery room resuscitation in excess of routine care; however, a small number of prenatally diagnosed cardiac lesions are more likely to require urgent postnatal intervention immediately following delivery. These cardiac lesions include transposition of the great arteries with intact ventricular septum and restrictive atrial septum, hypoplastic left heart syndrome with intact atrial septum, obstructed total anomalous pulmonary venous return, and complete congenital heart block. Prenatal diagnosis allows for coordination of care surrounding delivery and during the early postnatal hours.
Although congenital complete heart block (CHB) is usually well tolerated by the developing fetus, it may occasionally result in hydrops fetalis1,2 with a significant risk of fetal or neonatal death.3 The treatment of the fetus with CHB and hydrops remains a difficult problem. Attempted pharmacologic therapies have included β-adrenergic agonists,3 corticosteroids,4 and intravenous immunoglobulins, 5 none of which have been widely effective. One report of transuterine transthoracic fetal ventricular pacing was not successful in bringing the fetus to term.6 Although animal studies of fetal permanent pacemaker systems implantation are under way,7 this approach has not been used in humans. Because currently available in utero therapy is unable to halt the progression of fetal hydrops, one must usually resort to early delivery and extrauterine pacing of a compromised (and often premature) neonate. On occasion, these infants are too small to accept immediate implantation of a permanent pacing system, and use of temporary transvenous pacing wires carries the risk of cardiac perforation and venous thrombosis in the very small child. This report describes an alternate pacing strategy in 2 premature infants with CHB, involving the use of temporary epicardial pacing wires to bridge the interval until the permanent pacemaker can be implanted.
Evaluation of fetal bradycardia noted during a routine prenatal visit at 24 weeks' gestation in a mother without symptoms showed an antibody-mediated heart block with anti-La (SSB) antibody present. 'The fetus had a pericardial effusion and ascites. After maternal steroid therapy, the ascites resolved and the pericardial effusion was substantially diminished. Steroid therapy may be a helpful adjunct for treatment of a premature infant with hydrops as a result of antibody-mediated heart block. (Am J Obstet Gynecol 1991;165:553-4.)
Fetal heart rate monitoring was combined with fetal echocardiography for examination of atrial reactivity during labour in five fetuses with second or third degree heart block. Alterations in vagal tone accompanying uterine contraction influence atrial rate, even when the ventricle is not under atrial 'control'. Fetal echocardiography enabled diagnosis of the underlying basis of the arrhythmia and located the optimal position for recording atrial activity with an external heart rate monitor. External and internal monitoring of atrial activity demonstrated reactivity during labour. Two patients were delivered vaginally after monitoring throughout labour. One mother preferred elective caesarean delivery. Caesarean section was required in another for cephalopelvic disproportion and in the remaining woman for late decelerations. These monitoring techniques provide an assessment of fetal well-being in the presence of fetal bradycardia due to variable degrees of heart block.