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. 2010 Aug-Sep;26(7):371-6.
doi: 10.1016/s0828-282x(10)70418-9.

Cor triatriatum sinistrum in childhood. A single institution's experience

Tilman Humpl  1 , Katja Reineker, Cedric Manlhiot, Anne I Dipchand, John G Coles, Brian W McCrindle
Affiliations

Cor triatriatum sinistrum in childhood. A single institution's experience

Tilman Humpl et al. Can J Cardiol. 2010 Aug-Sep.

Abstract
in English, French

Introduction: Cor triatriatum sinistrum (CTS) is a rare congenital cardiac defect that can present with a wide range of symptoms and may be associated with other structural cardiac defects. Very limited data are available for pediatric patients.

Objective: To analyze the experience with CTS at a single pediatric centre, highlighting symptoms on presentation, mode of diagnosis and outcome.

Methods: Hospital databases were searched to identify patients with CTS who were diagnosed between 1954 and 2005. Medical records with demographic data, clinical evaluation, diagnostic approach, interventions and autopsy results were reviewed.

Results: Between 1954 and 2005, 82 patients (43 female children, 52%) with CTS were diagnosed at the institution. Patients were born between 1951 and 2004, and the median age at presentation was eight months (range one day to 16.1 years). The majority of patients (77%) presented with associated cardiac lesions. Of 82 patients, 57 (70%) underwent resection of the fibromuscular diaphragm, 14 (17%) did not require surgery and 11 (13%) did not survive the time to intervention. Nineteen patients (23%) died a median of two months (range one day to 5.5 years) after presentation. Nine of these patients (11%) died before surgery was attempted. Most patients were completely asymptomatic at the time of the last follow-up at a median of three years (range one day to 18 years). Echocardiography significantly facilitated the diagnosis and subsequent management of patients with CTS.

Conclusions: Despite the diversity in presentation and complexity of associated lesions, the outcome for patients with CTS is favourable.

INTRODUCTION :: Le cœur triatrial (CTA) gauche est une anomalie cardiaque congénitale rare, parfois accompagnée de toute une gamme de symptômes et associée à d’autres anomalies cardiaques structurales. On dispose de très peu de données à son sujet chez les patients pédiatriques.

OBJECTIFS :: Analyser l’expérience acquise sur le CTA dans un centre pédiatrique en mettant en lumière les symptômes au moment de la consultation, le diagnostic et l’issue.

MÉTHODES :: Les auteurs ont interrogé les bases de données de l’hôpital afin de recenser les patients atteints de CTA ayant reçu leur diagnostic entre 1954 et 2005. Ils ont passé en revue les dossiers médicaux comprenant les données démographiques, l’évaluation clinique, l’approche diagnostique, les interventions et les résultats de l’autopsie.

RÉSULTATS :: Entre 1954 et 2005, 82 patients (43 fillettes, 52 %) ont reçu le diagnostic de CTA dans cet établissement. Les patients étaient nés entre 1951 et 2004 et l’âge médian au moment de la consultation initiale était de huit mois (entre 1 jour et 16,1 ans). La majorité des patients (77 %) présentaient des lésions cardiaques associées. Sur les 82 patients, 57 (70 %) ont subi une résection de la cloison fibromusculaire, 14 (17 %) n’ont pas eu besoin de chirurgie et 11 (13 %) n’ont pas survécu jusqu’à l’intervention. Dix-neuf patients (23 %) sont décédés après une période médiane de deux mois (entre 1 jour et 5,5 ans) suivant la consultation initiale. Neuf de ces patients (11 %) sont décédés avant que la chirurgie n’ait pu être tentée. La plupart des patients étaient entièrement asymptomatiques au moment du dernier suivi après une période médiane de trois ans (entre 1 jour et 18 ans). L’échocardiographie a grandement facilité le diagnostic et la prise en charge subséquente des patients atteints de CTA.

CONCLUSIONS :: Malgré la diversité et la complexité des lésions associées, l’issue est restée favorable chez les patients atteints de CTA.

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Figures

Figure 1)
Figure 1)
Survival of patients with cor triatriatum sinistrum after diagnosis. Era 1 includes all patients diagnosed between 1956 and 1982, and era 2 includes all patients diagnosed between 1983 and 2005
Figure 2)
Figure 2)
Survival of patients with cor triatriatum sinistrum since surgery. Era 1 includes all patients diagnosed between 1956 and 1982, and era 2 includes all patients diagnosed between 1983 and 2005
Figure 3)
Figure 3)
Changes over time of right ventricular systolic pressure (RVSP) after surgery in patients with cor triatriatum sinistrum. The solid line represents the estimate of change in RVSP over time and the dashed lines represent the 95% CIs. P values are the estimate of the statistical significance of the observed time trend
Figure 4)
Figure 4)
Change over time in z-score (size) for pulmonary and aortic valves (A), and mitral and tricuspid valves (B) measured by echocardiography after surgery. The solid lines represent the estimates of change in z-scores over time and the dashed lines represent the 95% CIs. P values are the estimates of the statistical significance of the observed time trends
Figure 5)
Figure 5)
Changes over time in right and left ventricular end-diastolic dimensions after surgery in patients with cor triatriatum sinistrum. The solid lines represent the estimates of change in z-scores over time and the dashed lines represent the 95% CIs. P values are the estimates of the statistical significance of the observed time trends
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