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Association of A Dilated Coronary Sinus in the Fetus with Actual and Apparent Coarctation of the Aorta and Diminutive Left Heart Structures

Ramaswamy, Prema; Rafii, Daniela; Osmolovsky, Marina; Agarwal, Arpit; Amirtharaj, Cynthia
Evidence suggests an association between left heart obstructive lesions and dilated coronary sinus (DCS), but this has not been studied in fetuses. A retrospective review of fetal echocardiograms (FE) over an 8-year period was conducted, and patients with DCS were identified and confirmed postnatally. There were 5840 FE performed on 4920 women during this period. Of 49 patients with DCS, 22 had normal intracardiac anatomy and 27 patients had congenital heart disease (CHD) yielding an incidence of 4.6 % in the presence of CHD (27/584). Of 27 patients with DCS and CHD, approximately a third had either hypoplastic left ventricles and/or coarctations (10/27, 37 %). The incidence of left heart obstructive lesions was much higher in the presence of a DCS (37 % vs 45/557, 8 %, p < 0.0001). The odds ratio of left heart hypoplasia in fetuses with CHD and a DCS was 6.6 (95 % CI 2.8-15.3). Comparison of patients with postnatally confirmed coarctation with those with normal intracardiac anatomy with DCS, revealed that in the former, the right ventricle (p = 0.005), pulmonic valve annulus (p = 0.0001) and the tricuspid inflow were larger (p = 0.001) compared to corresponding left-sided structures. The size of the DCS was not significantly different between the two groups, but in the former, the DCS was more closely related to the posterior leaflet of the mitral valve and caused a significant diminution of the mitral inflow. Our study suggests a strong association, possibly causal, between left heart obstructive lesions and DCS in utero.
PMID: 27558552
ISSN: 1432-1971
CID: 3098462

Fetal Diagnosis of Abnormal Origin of the Left Pulmonary Artery [Case Report]

Tretter, Justin T; Tretter, Eric M; Rafii, Daniela Y; Anderson, Robert H; Bhatla, Puneet
Isolated anomalies of the branch pulmonary arteries are rare, more often occurring in the setting of complex congenital heart disease. These isolated anomalies are often not identified in the prenatal period. We describe two cases of isolated anomalies of the left pulmonary artery which were identified on fetal echocardiography and confirmed postnatally, an anomalous left pulmonary artery arising from the base of the left-sided brachiocephalic artery in the setting of a right-sided aortic arch, and a left pulmonary artery sling. These two cases support our current understanding of normal and abnormal development of the extrapericardial arterial vessels and highlight the importance of meticulous attention when sweeping from the three-vessel tracheal view.
PMID: 27132629
ISSN: 1540-8175
CID: 2101042

Relationship of ambulatory blood pressure and body mass index to left ventricular mass index in pediatric patients with casual hypertension

Ramaswamy, Prema; Chikkabyrappa, Sathish; Donda, Keyur; Osmolovsky, Marina; Rojas, Mary; Rafii, Daniela
Both obesity and hypertension are associated with left ventricular hypertrophy (LVH) in children. Our objective was to compare the prevalence of LVH in obese and nonobese subjects with casual hypertension who underwent ambulatory blood pressure monitoring (ABPM). Untreated children (aged 6-20 years) underwent 24-hour ABPM, and left ventricular mass index (LVMI) was measured. Subjects were classified into three groups: white coat hypertension (WCH), prehypertension (pre-HT), and hypertension (HT). The prevalence of LVH was compared between obese and nonobese subjects among the groups. Of 69 children who underwent ABPM, thirty-two patients (46%) had WCH, 13 (19%) had pre-HT, and 24 (35%) had HT. Mean age, BMI, and LVMI were similar in the groups (P = not significant [NS]). In all, 22 patients (32%) had LVH, with no difference among WCH versus pre-HT versus HT (37.5% vs. 46% vs. 16.7%, P = NS). Twenty-seven subjects (39%) were obese. The ratio of LVH in obese to nonobese was 55.5% to 16.6% (P = .001). In both pre-HT and WCH, patients with LVH had a significantly higher BMI z score (P = .02 and P = .01, respectively). LVMI correlated strongly with BMI z score (P = .0001) but not with any blood pressure parameter. Almost half of children with casual HT have WCH. LVH is prevalent in a third of children with HT, pre-HT, and WCH. In both pre-HT and WCH, patients with LVH were more likely to be obese. More than half of all the obese subjects had LVH. Obese children in all three groups may be at a greater risk for end organ damage.
PMID: 26725015
ISSN: 1878-7436
CID: 2042462

Age less than two years is not a risk factor for mortality after mitral valve replacement in children

Rafii, Daniela Y; Davies, Ryan R; Carroll, Sheila J; Quaegebeur, Jan M; Chen, Jonathan M
BACKGROUND: Outcomes for mitral valve replacement (MVR) in the pediatric population are generally reported as poor, particularly in patients less than 2 years old. We compared long-term morbidity and mortality associated with MVR between patients less than 2 years old and patients 2 to 18 years old. METHODS: We evaluated pediatric patients undergoing MVR from March 1990 to November 2007 at our institution. Morbidity and mortality was compared between patients less than 2 years and patients 2 to 18 years old. Primary endpoints measured were postoperative survival, long-term survival, reoperation, cerebrovascular accident or transient ischemic attack, and significant bleeding events. RESULTS: Forty-five patients underwent 54 MVRs. Median age was 3.1 years; 18 patients were under 2 years. Median follow-up time was 5.4 years. There was no statistically significant difference between long-term or short-term survival between the two age groups, with 30-day survival of 89% (younger patients) versus 100% (older patients), and 10-year survival of 82% (younger patients) versus 85% (older patients). Freedom from reoperation for the younger age group was 40% at 10 years versus 96% for the older patients, p = 0.003. CONCLUSIONS: In our population, there was no statistically significant difference in survival between patients less than 2 years and patients 2 to 18 years. In children undergoing MVR, age less than 2 years remains a risk factor for reoperation but not for mortality
PMID: 21440150
ISSN: 1552-6259
CID: 140754