A case of a large leiomyomatous uterus with multiple arteriovenous malformations and subsequent high cardiac output state with severe four chamber cardiac enlargement

Abstract Introduction Little is known about cardiac involvement in thalassemia intermedia (TI) using cardiovascular magnetic resonance (CMR). We investigated myocardial iron overload (MIO), biventricular parameters, and myocardial fibrosis in a large cohort of TI patients, underlying the differences between transfusion-dependent and non-transfusion-dependent patients. Methods We studied 252 adult TI patients (119 females, 39.5±10.4 years) enrolled in the MIOT Network. MIO was assessed using a multislice multiecho T2* approach. Biventricular function parameters were quantified by cine sequences. Myocardial fibrosis was evaluated by late gadolinium enhancement acquisitions. Results One-hundred and eighty-eight (74.6%) patients showed no MIO in any segment, 56 (22%) had an heterogeneous distribution (52 with global heart T2*≥20 ms), and 8 (0.3%) showed an homogeneous MIO. Left ventricular (LV) and right ventricular (RV) dilatations were present in 113 (45%) and in 49 (19%) patients, respectively. LV dysfunction was present in the 18.0% of the cases while RV dysfunction in the 3.63%. High LV mass indexes were present in 22 (8.7%) patients. Fifty-two/227 (22.9%) patients showed myocardial fibrosis. Myocardial fibrosis was associated to LV dysfunction (P=0.001) and high mass indexes (P=0.038). One-hundred and fourteen patients were non-transfusion dependent (transfusion requirements absent or sporadic) while 138 patients were transfusion-dependent (regular transfusions). The mean age at start of chronic transfusions was 11.8 ± 12.3 years. Table 1 shows the comparison between the two groups. Non-transfusion-dependent patients showed significantly higher global heart T2* values and MIO with a global heart T2* < 20 ms was detected in two of them (one requiring occasional blood transfusions and one non transfused). Biventricular end-diastolic volume index, stroke volume index, left ventricular (LV) mass index, and LV cardiac index were significantly higher in the non-transfusion dependent group. Conclusions CMR plays a key role in the management of TI patients. Heart iron (global heart T2* < 20 ms) was not common, but a quarter of the patients had some pathological segments. A consistent number of patients had the stigmata of the high cardiac output state cardiomyopathy. Myocardial fibrosis was related to the high cardiac output state. The signs of the high output state were controlled in the transfusion-dependent-patients. Disclosures: No relevant conflicts of interest to declare.

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Giant Chorioangiomas: Perinatal Outcomes and Techniques in Fetoscopic Devascularization

Fetal Diagnosis and Therapy ◽

10.1159/000363600 ◽

2014 ◽

Vol 37 (1) ◽

pp. 18-23 ◽

Cited By ~ 10

Author(s):

Foong-Yen Lim ◽

Alan Coleman ◽

William Polzin ◽

Ronald Jaekle ◽

Mounira Habli ◽

...

Keyword(s):

Cardiac Output ◽

Perinatal Outcomes ◽

Bipolar Coagulation ◽

High Cardiac Output ◽

Output State ◽

Obstetrical Complications ◽

Surgical Clip ◽

Clip Application ◽

Arterial Inflow ◽

Prenatal Imaging

Objective: To evaluate the intrauterine management and perinatal outcome of pregnancies complicated by giant placental chorioangioma (>4 cm) and elaborate on various devascularization techniques. Materials and Methods: Retrospective review of 10 cases of giant placental chorioangioma evaluated between January 2005 and August 2012. Maternal demographics, prenatal imaging, response to fetoscopic treatment, obstetrical complications, and perinatal outcomes were evaluated. Results: Overall survival was 80%. Seven (70%) cases were associated with obstetrical complications, including polyhydramnios (n = 7), non-immune hydrops (n = 3), and high cardiac output state (n = 5). Five patients underwent fetoscopic devascularization at a mean gestational age of 24.03 weeks with 80% survival. The tumors were devascularized by bipolar coagulation (n = 1), combination of bipolar and diode laser (n = 2), bipolar and radiofrequency ablation (n = 1), and surgical clip application (n = 1). Postoperatively, all survivors had resolution of hydrops and high cardiac output states with survival beyond 60 days of life. Discussion: Fetoscopic devascularization is indicated for high cardiac output states or non-immune hydrops and may require multiple techniques including bipolar coagulation, clip application, and/or laser to interrupt arterial inflow and devascularize the mass.

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