Denise Donaghue

Perinatal and early surgical outcome for the fetus with hypoplastic left heart syndrome: a 5-year single institutional experience.

To review our experience with the prenatal diagnosis of hypoplastic left heart syndrome (HLHS). Our goal was to establish the benchmark for perinatal and early surgical outcome in the current era, from a center with an aggressive surgical approach and a cohort with a high level of intention‐to‐treat.

Vasoreactive response to maternal hyperoxygenation in the fetus with hypoplastic left heart syndrome.

Background—Cardiopulmonary interactions play an important role in the pathophysiology of hypoplastic left heart syndrome (HLHS). Pulmonary vasculopathy has been identified, especially in those with restrictive/intact atrial septum. Responsiveness of the pulmonary vasculature to maternal hyperoxygenation (MH) may provide a tool to assess the degree of pulmonary vasculopathy present before birth. Methods and Results—Doppler echocardiography was performed in 27 normal and 43 HLHS fetuses. In HLHS, sampling was repeated after 10 minutes of MH with 60% FiO2 and after 5 minutes of recovery. Sampling was performed in the proximal, midportion, and distal branch pulmonary artery (PA). Pulsatility index (PI) was used as a measure of vascular impedance. Of the HLHS fetuses, 34 had an open interatrial septum and 9 had a restrictive/intact atrial septum. At birth, 5 fetuses underwent immediate intervention on the interatrial septum. Middle cerebral artery PI was lower in HLHS versus normal fetuses (P<0.001). There was no difference in UA, DA, or branch PA PI between normal fetuses and those with HLHS. MH led to a significant decrease in PI at each of the PA sites sampled in fetuses with an open atrial septum (P<0.001); however, there no was significant change in the PI in fetuses that required immediate intervention on the atrial septum at birth. Using a cutoff value of <10% vasoreactivity, the sensitivity of MH testing for determining need for immediate intervention at birth is 100% (0.46 to 1.0); specificity, 94% (0.78 to 0.99); positive predictive value, 71% (0.30 to 0.95); and negative predictive value, 100% (0.86 to 1.0). No untoward effects were seen with MH. Conclusions—PA vasoreactivity to MH occurs in the fetus with HLHS. MH testing accurately identifies fetuses requiring urgent postnatal intervention at birth and may be used to select candidates for fetal atrial septoplasty.

Right Ventricular Performance in the Fetus With Hypoplastic Left Heart Syndrome

BACKGROUND In the fetus with hypoplastic left heart syndrome (HLHS), the single right ventricle (RV) pumps the entire cardiac output in utero. By investigating RV performance in utero, we sought to determine the inherent capabilities of a single RV before the increased metabolic demands of postnatal life and surgical palliation. In addition, we sought to determine whether the presence or absence of a left ventricular cavity impacts on RV performance in fetal life. METHODS Between November 2004 and December 2006, Doppler flow-derived measures of ventricular performance were obtained with echocardiography in 76 fetuses with normal cardiovascular system and in 48 age-matched fetuses with HLHS from 17 weeks until 40 weeks of gestation. The myocardial performance index, ventricular ejection force, and cardiac output were determined for both groups and compared using unpaired Students t tests and regression analysis. RESULTS In fetuses with HLHS, cardiac output was diminished by 20%, RV ejection force was elevated, and RV myocardial performance index was elevated compared with those of normal fetuses. The presence of a left ventricular cavity did not impact on RV performance in utero. CONCLUSIONS Fetuses with HLHS have preserved systolic performance but impaired diastolic performance compared with normal fetuses. The heart of a fetus with HLHS is less efficient than the normal heart in that ejection force of the RV is increased, but overall delivery of cardiac output is lower than normal. We conclude that patients with HLHS have inherent limitations in cardiac performance even before birth.

Impact of altered loading conditions on ventricular performance in fetuses with congenital cystic adenomatoid malformation and twin-twin transfusion syndrome

In the fetus with a structurally normal heart, two conditions—giant chest mass, such as congenital cystic adenomatoid malformation (CCAM), and twin–twin transfusion syndrome (TTTS)—alter ventricular loading conditions and may result in cardiovascular compromise. The aim of this study was to elucidate the mechanism of cardiovascular dysfunction by comparing geometry‐independent, Doppler flow‐derived measures of ventricular performance in fetuses with altered loading conditions vs. those in normal fetuses.

Parental decision-making in congenital heart disease.

OBJECTIVE To explore whether prenatal diagnosis of congenital heart disease is associated with lower levels of parental distress and greater satisfaction with decisions about cardiothoracic surgery when compared to postnatal diagnosis. METHODOLOGY A combined quantitative-qualitative design was used. Participants included the parents of 31 neonates (30 mothers and 22 fathers) admitted to the cardiac intensive care unit between 1 November 2001 and 1 May 2002 for repair of congenital cardiac malformations. Participants completed self-report measures of anxiety, optimism, and life events pre-operatively, and semi-structured qualitative interviews assessing satisfaction with decision-making within 1 week of the operation. RESULTS At the time of surgery, mothers of neonates receiving the diagnosis prenatally did not differ from mothers of neonates receiving the diagnosis postnatally on measures of anxiety, optimism, and life events. Fathers of neonates receiving the diagnosis prenatally, however, reported more optimism, lower state and trait anxiety, and fewer negative life events than fathers of neonates receiving the diagnosis postnatally. When we analyzed the interviews, we found that, regardless of the timing of the diagnosis, parents felt as though they made a genuine choice for their baby to have surgery. CONCLUSIONS In this pilot study, fathers who learned prenatally that their child had a congenital cardiac malformation were less distressed than those who discovered this fact only postnatally. From the parental perspective, nonetheless, distress and urgency do not impair their ability to make decisions about neonatal cardiac surgery.

Left ventricle to right ventricle size discrepancy in the fetus: the presence of critical congenital heart disease can be reliably predicted.

BACKGROUND Prenatal ventricular size discrepancy with disproportionately smaller left ventricle than right ventricle (L-R/VD) can be a marker for important left-sided structural heart disease in the newborn. METHODS We reviewed the echocardiograms of all fetuses evaluated at our center with L-R/VD from July 1, 2004 to January 1, 2008. RESULTS Of the 35 fetuses, 20 (57%) had critical arch obstruction and underwent neonatal intervention (group 1); 15 (43%) did not require newborn intervention (group 2). Ratios comparing left with right heart structures were significantly lower in group 1 fetuses compared with group 2 fetuses. Aortic arch measurement <or= 3 mm was the most sensitive, and abnormal direction of atrial level shunting was the most specific measure to predict the need for neonatal aortic arch intervention. CONCLUSION Ratios expressing the magnitude of L-R/VD, direction of flow at the atrial septum, and measurements of the aortic arch help identify fetuses that will require neonatal intervention.

Fetal pulmonary venous Doppler patterns in hypoplastic left heart syndrome: relationship to atrial septal restriction

Objective: Pulmonary venous Doppler (PVD) patterns are abnormal in fetuses with hypoplastic left heart syndrome (HLHS) with restricted foramen ovale (rFO) when compared with healthy fetuses. The objective of this study was to define PVD patterns in HLHS fetuses with an unrestricted or patent foramen ovale (pFO). Design: 27 fetuses with HLHS and 66 healthy fetuses underwent echocardiography between 19 and 38 weeks of gestation. The pulmonary venous peak systolic (S), diastolic (D) and atrial reversal (A) velocities were measured; S/D ratio, velocity time integral of forward (VTIf) and reversed (VTIr) flows and VTIr expressed as percentage of VTIf (%R) were calculated. Independent examiners classified HLHS subjects into HLHS-pFO and HLHS-rFO (rFO or intact atrial septum). Setting: Tertiary referral centre for paediatric cardiology. Results: Compared with healthy controls, the HLHS-pFO group (n = 16) had higher S (32.9 (3.2) vs 23.5 (1.6) cm/s (adjusted mean (SE)); p = 0.01), A (10.5 (3.4) vs 0.17 (1.6) cm/s; p = 0.01), VTIr (0.78 (0.3) vs 0.01 (0.13) cm; p = 0.01) and %R (14.2% (3.2 %) vs −1.3% (1.5%); p<0.001). D velocity, S/D and VTIf showed no difference. In HLHS-rFO, further increase in S, A, VTIr and %R, decrease in D, increase in S/D and no change in VTIf were noted. Conclusions: PVD flow patterns are abnormal in HLHS even in the absence of rFO, suggesting that factors other than impaired left atrial egress play a role. Future studies of PVD patterns can provide important insights into left atrial dynamics, pulmonary venous return and pulmonary vascular development in fetal HLHS.

Anatomic variability and outcome in prenatally diagnosed absent pulmonary valve syndrome.

BACKGROUND We sought to describe current outcomes and risk factors for mortality for fetuses diagnosed with absent pulmonary valve syndrome (APV). Fetuses with APV were divided into two cohorts, those with underlying tetralogy of Fallot (TOF/APV) and those without underlying TOF and either an intact ventricular septum or small ventricular septal defect (APV/IVS). METHODS The fetal echocardiographic database was reviewed from January 1, 2001, until June 1, 2010, and all subjects with a diagnosis of APV were included. Multiple clinical and fetal echocardiographic measurements were recorded. Statistical analysis was performed by χ2 analysis and t tests. Survival analysis was performed by Kaplan-Meier analysis. Significant relationships between variables were explored by regression analysis. Significance was set at p=0.05. RESULTS The cohort consisted of 15 fetuses with TOF/APV and 6 fetuses with APV/IVS. There were no fetal demises in either cohort. Survival to birth was 71% in the TOF/APV cohort and 83% in the APV/IVS cohort (p=0.62). Of subjects born alive, survival was 80% for both cohorts (p=0.95). However, in the APV/IVS cohort, transplantation-free survival was only 20%. Underlying single-ventricle physiology strongly predicted those who underwent heart transplantation (p=0.003, R2=0.50). For the entire APV cohort, left ventricular dysfunction (p=0.005, R2=0.41) and a higher pulmonary artery valve-to-aortic valve ratio (p=0.02, R2=0.34) predicted mortality. CONCLUSIONS Postnatal outcomes continue to improve for fetuses with APV syndrome. Left ventricular dysfunction and higher pulmonary artery valve-to-aortic valve ratio accurately predict postnatal mortality for fetuses with APV.

OC108: Ventricular performance in the fetus with hypoplastic left heart syndrome

DOI:€10.1016/j.athoracsur.2008.11.032 Ann Thorac Surg 2009;87:1214-1219 Anita Szwast, Zhiyun Tian, Margaret McCann, Denise Donaghue and Jack Rychik Syndrome Right Ventricular Performance in the Fetus With Hypoplastic Left Hearthttp://ats.ctsnetjournals.org/cgi/content/full/87/4/1214 located on the World Wide Web at: The online version of this article, along with updated information and services, is

1057-206 Fetal pulmonary venous doppler patterns in hypoplastic left heart syndrome: Relationship to atrial septal restriction

OBJECTIVE Pulmonary venous Doppler (PVD) patterns are abnormal in fetuses with hypoplastic left heart syndrome (HLHS) with restricted foramen ovale (rFO) when compared with healthy fetuses. The objective of this study was to define PVD patterns in HLHS fetuses with an unrestricted or patent foramen ovale (pFO). DESIGN 27 fetuses with HLHS and 66 healthy fetuses underwent echocardiography between 19 and 38 weeks of gestation. The pulmonary venous peak systolic (S), diastolic (D) and atrial reversal (A) velocities were measured; S/D ratio, velocity time integral of forward (VTI(f)) and reversed (VTI(r)) flows and VTI(r) expressed as percentage of VTI(f) (%R) were calculated. Independent examiners classified HLHS subjects into HLHS-pFO and HLHS-rFO (rFO or intact atrial septum). SETTING Tertiary referral centre for paediatric cardiology. RESULTS Compared with healthy controls, the HLHS-pFO group (n = 16) had higher S (32.9 (3.2) vs 23.5 (1.6) cm/s (adjusted mean (SE)); p = 0.01), A (10.5 (3.4) vs 0.17 (1.6) cm/s; p = 0.01), VTI(r) (0.78 (0.3) vs 0.01 (0.13) cm; p = 0.01) and %R (14.2% (3.2 %) vs -1.3% (1.5%); p<0.001). D velocity, S/D and VTI(f) showed no difference. In HLHS-rFO, further increase in S, A, VTIr and %R, decrease in D, increase in S/D and no change in VTI(f) were noted. CONCLUSIONS PVD flow patterns are abnormal in HLHS even in the absence of rFO, suggesting that factors other than impaired left atrial egress play a role. Future studies of PVD patterns can provide important insights into left atrial dynamics, pulmonary venous return and pulmonary vascular development in fetal HLHS.