Isolated congenital atrioventricular block diagnosed in utero: natural history and outcome

Johannes M P J Breur1, Livia Kapusta, Philip Stoutenbeek

  • 1Department of Obstetrics, University Medical Center, Utrecht, the Netherlands. hans_breur@hotmail.com

Insights

Congenital atrioventricular block (CAVB) diagnosed in utero has high mortality, especially with fetal hydrops or heart rates below 50 bpm. Poor outcomes, including pacemaker implantation or dilated cardiomyopathy, are linked to fetal heart rates below 60 bpm.

Area of Science:

  • Cardiology
  • Fetal Medicine
  • Pediatric Cardiology

Background:

  • Isolated congenital atrioventricular block (CAVB) diagnosed in utero presents significant morbidity and mortality risks.
  • Prognosis is particularly poor with fetal heart rates below 55 beats per minute (bpm) or the development of fetal hydrops.

Purpose of the Study:

  • To describe the natural history and outcomes of 24 infants with isolated CAVB diagnosed prenatally.
  • To review existing literature on CAVB.
  • To identify risk factors predicting outcomes in isolated CAVB.

Main Methods:

  • Retrospective multicenter study involving 24 patients diagnosed with isolated CAVB in utero.
  • Analysis of fetal heart rates, gestational age at diagnosis, development of fetal hydrops, and subsequent infant outcomes.

Main Results:

  • CAVB was detected at a mean gestational age of 24.7 weeks; 10 fetuses presented with complete heart block initially.
  • Fetal hydrops occurred in 42% of cases, associated with lower heart rates (47 bpm vs. 57 bpm).
  • Mortality was 21%, linked to heart rates <50 bpm and fetal hydrops; 83% experienced poor outcomes (death, pacemaker, or dilated cardiomyopathy) associated with heart rates <60 bpm.

Conclusions:

  • Isolated CAVB diagnosed in utero carries a high risk of morbidity and mortality.
  • Fetal hydrops and lower fetal heart rates (<50 bpm) are associated with increased mortality.
  • Fetal heart rates below 60 bpm predict the need for pacemaker implantation and/or the development of dilated cardiomyopathy.
Abstract

Related Concept Videos

Dysrhythmias IV: Characteristics of Bradyarrhythmias01:18

Dysrhythmias IV: Characteristics of Bradyarrhythmias

Bradyarrhythmias are cardiac rhythm disorders characterized by a slower-than-normal heart rate, typically defined as fewer than 60 beats per minute. Some of which are discussed here:Sinus BradycardiaSinus bradycardia presents a heart rate lower than 60 beats per minute, with a regular rhythm originating from the SA node. The ECG typically shows normal P waves preceding each QRS complex, a normal PR interval (0.12 to 0.20 seconds), and a normal QRS duration (0.06 to 0.10 seconds).First-Degree AV...
Mitral Stenosis II: Clinical features and Diagnostic Tests01:23

Mitral Stenosis II: Clinical features and Diagnostic Tests

Mitral stenosis is a heart condition in which the mitral valve, which allows blood to flow from the left atrium to the left ventricle, becomes narrowed or stenotic. This narrowing hinders blood flow and leads to clinical symptoms requiring specific medical evaluations and management strategies. The following overview outlines the clinical symptoms, assessments, diagnostic findings, prevention methods, and treatments for mitral stenosis.Clinical ManifestationsDyspnea (shortness of breath): This...
Mitral Stenosis I: Introduction01:22

Mitral Stenosis I: Introduction

Mitral Valve Stenosis (MVS) is a heart condition where the mitral valve narrows, impeding blood circulation from the left atrium to the left ventricle. The etiology and pathophysiology of this condition are multifaceted, leading to a cascade of cardiovascular complications.Causes of Mitral Valve StenosisRheumatic Heart Disease: It is the main cause of mitral valve stenosis, particularly in developing nations. This condition arises from rheumatic fever, an inflammatory illness resulting from...
Development of the Heart01:27

Development of the Heart

The development of the human heart, a crucial organ, commences from the mesoderm on the 18th or 19th day after fertilization. This process initiates in the cardiogenic area, a group of mesodermal cells at the embryo's head end, which evolves into elongated strands known as cardiogenic cords. These cords undergo a transformation to form hollow-centered endocardial tubes.
As the embryo undergoes lateral folding, these paired tubes approach each other, merging into a single primitive heart tube by...
Mitral Regurgitation I: Introduction01:20

Mitral Regurgitation I: Introduction

Mitral regurgitation is characterized by the backward circulation of blood from the left ventricle to the left atrium during systole, a phase of the cardiac cycle when the heart contracts and pumps blood out of the chambers. This abnormal flow occurs primarily due to the dysfunction of the mitral valve or its supporting structures, which include the mitral leaflets, chordae tendineae, annulus, and papillary muscles.Etiology and Mechanisms:Primary Mitral Regurgitation: This type arises from...
Mitral Valve Prolapse I: Introduction01:27

Mitral Valve Prolapse I: Introduction

IntroductionThe mitral valve, one of the heart's four valves, regulates blood flow. These valves have flaps that open and close to direct blood properly through the heart and body. During each heartbeat, the flaps open for blood to pass through and seal shut to prevent backflow. Specifically, the mitral valve opens to allow blood flow from the heart's upper left chamber to the lower left chamber. It then closes securely as the lower left chamber contracts to pump blood to the body, preventing...