Changes in echocardiography and blood variables during and after development of Ballantyne syndrome

Abstract

We report a pregnant woman who was monitored by echocardiography and determination of blood variables, including components of the renin-angiotensin-aldosterone system (RAAS), cardiac biomarkers and soluble fms-like tyrosine kinase-1 (sFlt-1), during and after the development of Ballantyne syndrome. Generalised maternal oedema with dyspnoea following fetal and placental hydrops necessitated a caesarean section at 33 weeks of gestation. Changes in blood variables and simultaneous echocardiography changes indicated acutely enhanced RAAS and hyperdynamic left ventricular function in response to excessive volume overload (as evidenced by brain-type natriuretic peptide level of 523 pg/mL) in the absence of increased systemic vascular resistance. Elevated sFlt-1 (15 600 pg/mL) and human chorionic gonadotrophin (404 000 IU/L) levels were also noted. The increased plasma aldosterone concentration (2070 pg/mL) may have been responsible for the increase in circulating plasma volume, and the increased sFlt-1 level was responsible for generalised maternal oedema. It remains unclear which factor(s) triggered RAAS activation.

Echocardiography findings in a case with Ballantyne syndrome

“…1, left) occurred in the presence of rather lower systemic vascular resistance compared to the data reported previously by Simmons et al. 9 All of these findings were consistent with those in the previous case with Ballantyne syndrome 3 suggesting that heart failure is common in this syndrome. Acute change in these echocardiography findings in a short time (within a week) may have been associated with an acute increase in circulating plasma volume.…”

“…3 In the present case, increased BNP in response to cardiac overload (as evidenced by increased LAVI and IVCD), hyperdynamic left ventricular function (as evidenced by increased LVEF and cardiac output), and pulmonary congestion (as evidenced by acute increase in PASP) (Fig. 1, left) occurred in the presence of rather lower systemic vascular resistance compared to the data reported previously by Simmons et al.…”

“…This explains increased interstitial fluid, i.e., edema, but not increased plasma volume in Ballantyne syndrome. The activated RAAS with increased PAC level is considered to be responsible for the increased plasma volume in pregnancy 4 and PAC was indeed markedly elevated in our previous case with Ballantyne syndrome, 3 but not in the present case. PAC can be released by hCG directly via stimulation of the luteinizing hormone receptor expressed in the adrenal gland.…”

“…3 Gestational weight gain may vary according to ethnicity. However, among 128,838 Japanese women with singleton pregnancies, 10 the mean (SD) weekly weight gain was 0.26 (0.12) kg and total gestational weight gain was approximately 9.6 (4.4) kg.…”

“…To our knowledge, however, there has been only one report regarding cardiac morphology and function determined on echocardiography in patients with Ballantyne syndrome. 3 The endocrine environment is markedly altered after pregnancy; the renin-angiotensin-aldosterone system (RAAS) is activated and plays a critical role in the increased circulating blood volume in pregnancy 4 and soluble fms-like tyrosine kinase-1 (sFlt-1) derived mainly from the placenta may be a key factor responsible for increased vascular permeability 5 leading to edema in pregnancy. Recently, we encountered a case of Ballantyne syndrome in which determinations of cardiac status on echocardiography and blood variables pertinent to RAAS as well as sFlt-1 were performed simultaneously.…”