Isolated cor triatriatum sinistrum and pregnancy: case report and review of the literature

Abstract

Purpose Isolated cor triatriatum sinistrum (CTS) is a heart malformation in which a perforated fibromuscular membrane divides the left atrium into two chambers. When communication between these chambers is restricted, a patient may have signs and symptoms of mitral stenosis. The later stages of pregnancy are associated with tachycardia and increases in intravascular volume. We describe how this altered physiology may affect pregnant women with asymptomatic CTS. We also review the literature relating to pregnancy in patients with CTS.Clinical features A 30-yr-old primigravida, at 40 weeks of gestation with pre-pregnancy diagnosed asymptomatic CTS, was admitted for delivery. She had no cardiac symptoms during pregnancy, and her vaginal delivery under epidural analgesia was uneventful. This cardiac malformation is infrequently described in pregnant women, but a literature review showed that the physiology of late pregnancy with increases in hemodynamic variables may result in cardiac decompensation. Conclusion While our patient with isolated CTS and an unrestrictive intra-atrial membrane had an asymptomatic pregnancy and an uneventful labour, the literature review suggests that the increase in intravascular volume and heart rate that occurs during late pregnancy and after delivery may result in cardiac decompensation, even in patients with asymptomatic CTS. RésuméObjectif Le coeur triatrial gauche (CTG) isole´est une malformation cardiaque dans laquelle une membrane fibromusculaire perfore´e divise l'oreillette gauche en deux chambres. Lorsque la communication entre ces chambres est restreinte, le patient pourrait pre´senter les signes et symptômes d'une ste´nose mitrale. En fin de grossesse, on peut observer de la tachycardie et des augmentations du volume intravasculaire. Nous de´crivons la façon dont cette physiologie modifie´e pourrait affecter les femmes enceintes atteintes de CTG asymptomatique. Nous passons e´galement en revue la litte´rature portant sur la grossesse chez les patientes atteintes de CTG. É léments cliniques Une primigeste de 30 ans, a`40 semaines de grossesse et chez laquelle un diagnostic de CTG asymptomatique avait e´te´pose´avant la grossesse, a e´te´admise a`l'hôpital pour l'accouchement. Pendant la grossesse, elle n'a pre´sente´aucun symptôme cardiaque, et Drs Bojanić and Bursać contributed equally to this work.

Cor triatriatum sinistrum diagnosed in the adulthood: a systematic review

“…In two patients, the diagnosis was established by autopsy 8 9. CTS was diagnosed during pregnancy in 10 patients10–19 and in 1 after successful labour 20. Obstructive membrane physiology was reported in 70 (41%) patients.…”

“…Whereas CTS in paediatric patients rarely is an incidental finding,29–33 40 this accounts for one of four adult patients with the non-obstructive membrane 15 17 21 47–4929.…”

“…Physiological cardiovascular changes occurring in pregnancy can increase pulmonary venous pressure and lead to clinical symptom presentation or exacerbation during pregnancy10–18 20 w63 in patients with CTS. A few cases of pulmonary oedema during pregnancy and labour have also been reported in young adult women having undiagnosed CTS 16 20…”

Anesthesia and cor triatriatum

“…[20][21][22][23][24][25][26][27] In these women the aperture across the accessory atrial membrane, adequate for a pre-pregnancy hemodynamic state, may become inadequate to accommodate large physiologic increases in hemodynamics associate with uterine autotransfusion after delivery. [28] Pre-operative treatments Dependent on presenting symptomatology of CT, a corrective surgery or rarely percutaneous balloon dilatation of intra-atrial aperture [29] may be indicated before major elective surgery. Pre-operatively, all symptomatic patients with CT should be medically treated and special emphasis should be placed on controlling of the fast ventricular rate to promote adequate diastolic filling.…”

Cor Triatriatum: A Review

“…48 Pregnancy and postpartum period induced hyperdynamic circulation precipitate symptomatology. 47,[49][50][51][52][53] The proximal chamber dilates similar to the left atrium as in mitral stenosis and, therefore, becomes a nidus for atrial arrhythmia and the harbinger of thrombus and ectopic foci resulting in cerebral and systemic embolic events. [54][55][56][57][58][59] Chronic anticoagulation therapy is mandatory in such circumstances.…”