Outcomes of Atrial Arrhythmia Surgery in Patients With Congenital Heart Disease: A Systematic Review

Outcomes of Atrial Arrhythmia Surgery in Patients With Congenital Heart Disease: A Systematic Review

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Houck CA, de Groot NMS, Kardys I, Niehot CD, Bogers AJJC, Mouws EMJP.
J Am Heart Assoc. 2020 Oct 20;9(19):e016921. doi: 10.1161/JAHA.120.016921. Epub 2020 Sep 25.
PMID: 32972303 Free article.

Take Home Points:

  • Bi-atrial arrhythmia surgery appears to be the strategy of choice for congenital heart disease patients with atrial fibrillation (whether paroxysmal or non-paroxysmal).
  • Recurrence of atrial tachyarrhythmia after bi-atrial arrhythmia surgery is in the order of 20% based on available follow up.
  • Right atrial arrhythmia surgery alone appears sufficient for patients with a history of only macro-reentrant atrial tachycardias.
  • To date, there are no published data to support isolated left atrial lesions for patients with congenital heart disease that are to undergo arrhythmia surgery.

Dr Jeremy P Moore

Commentary by Dr. Jeremy Moore (Los Angeles) Congenital and Pediatric Cardiac EP section editor:  This is a systematic review of the existing literature (1994-2019) on arrhythmia surgery among patients with congenital heart disease, as compiled by the Erasmus group in the Netherlands. Accordingly, the authors selected 28 studies after reviewing 2175 abstracts and 132 full-text articles. The authors categorized the types of arrhythmia surgery as 1) bi-atrial, 2) right-atrial only or 3) left-atrial only. The quality of the included studies was considered to generally be good and was compromised mostly by lack of information regarding follow up (usually when the patients of interest were included as part of a larger cohort).

Of the studies included, the overall atrial tachyarrhythmia (ATA) recurrence after arrhythmia surgery was 13%. The majority (18, 64%) of studies provided a comprehensive description of the lesion sets used in the arrhythmia surgery. Unfortunately, the methods for performing arrhythmia surgery differed significantly among studies and even within studies.

Bi-atrial arrhythmia surgery (n=19)

In most studies that included bi-atrial arrhythmia surgery (16/19), patients had a history of atrial fibrillation (AF). Fourteen studies reported outcomes specifically after bi-atrial arrhythmia surgery. Overall, ATA recurred in 13% of patients (IQR 0-27%) and, when considering only the larger studies (> 8 patients), the reported ATA recurrence was higher at 20% (IQR 11-39%) during follow-up ranging from 1.0 to 7.4 years (see forest plot below).

right sided arrhythmia surgery

Right-sided arrhythmia surgery (n=19)

For most of the modern studies, lesions were placed according to the right-sided Maze procedure as first proposed by Theodoro in 1998. The indication for right-sided arrhythmia surgery was AF (6), MRAT (1), both AF and MRAT (9), or prophylactic (3). Twelve studies specifically reported outcomes after right-sided arrhythmia surgery for patients with MRAT and/or AF. The median recurrence for AT/AF after right-sided arrhythmia surgery was 19% (IQR 7-29%).

Three studies directly compared bi-atrial to right-sided only Maze procedures (studies by Stulak, Im, and Kobayashi and colleagues), the latter two including only ASD patients. Among all 3 studies, recurrence appeared to be higher for right-sided only Maze procedures that were performed for patients with pre-operative AF or longstanding ATA.

Left-sided arrhythmia surgery only (n=5)

These procedures were uncommonly performed and were isolated to subpopulations within larger studies. None of the publications reported outcomes separately.

Specific congenital heart disease defects

Among studies examining specific forms of congenital heart disease, bi-atrial surgery appeared more effective than right-atrial surgery for the reduction of ATA recurrence among patients with either ASD or Ebstein’s anomaly. On the other hand, only right atrial arrhythmia surgery was reported among patients with tetralogy of Fallot in one study and was associated with lower ATA recurrence as compared to isolated surgical repair.

The data presented in this systematic review were inherently limited in light of the existing literature. As the authors rightly point out, most studies to date have been characterized by small sample size and resultant wide confidence bounds. The indication for arrhythmia surgery have been inconsistently stated, the surgical approach (and energy source) have varied within or across studies and follow up has been limited or not reported.  As a result, the strength of the evidence to guide specific types of arrhythmia surgery in various settings remains weak. That said, basic principles as outlined above can be used to guide the surgical approach among patients with congenital heart disease.