scholarly journals The impact of prenatal counselling on mothers of surviving children with hypoplastic left heart syndrome: A qualitative interview study

2020 ◽  
Vol 23 (5) ◽  
pp. 1224-1230
Author(s):  
Sophie Bertaud ◽  
David F. A. Lloyd ◽  
Gurleen Sharland ◽  
Reza Razavi ◽  
Myra Bluebond‐Langner
Keyword(s):  
Hypoplastic Left Heart Syndrome ◽  
Qualitative Interview ◽  
Interview Study ◽  
Left Heart ◽  
Hypoplastic Left Heart ◽  
Prenatal Counselling ◽  
Qualitative Interview Study ◽  
Left Heart Syndrome ◽  
The Impact

scholarly journals Geometry of the pulmonary arteries before the Fontan operation: can we influence it during the Norwood procedure?

2020 ◽  
Vol 57 (6) ◽  
pp. 1098-1104
Author(s):  
Katarzyna Januszewska ◽  
Pawel Nawrocki ◽  
Anja Lehner ◽  
Julia Stegger ◽  
Felix Kleinerueschkamp ◽  
...  
Keyword(s):  
Hypoplastic Left Heart Syndrome ◽  
Fontan Operation ◽  
Pulmonary Arteries ◽  
Norwood Procedure ◽  
Left Heart ◽  
Hypoplastic Left Heart ◽  
Second Stage ◽  
The Right ◽  
Left Heart Syndrome ◽  
The Impact

Abstract OBJECTIVES The right ventricle-to-pulmonary artery (RV-PA) shunt provides stable haemodynamics after the Norwood procedure but can influence development of the central pulmonary arteries (PAs). The goal of this study was to analyse the geometry of the central PAs in children with hypoplastic left heart syndrome before the Fontan operation with respect to the RV-PA shunt site and the type of the second-stage operation. METHODS A total of 161 children with hypoplastic left heart syndrome, median age 2.7 (range 1.3–9.8) years and median weight 12.7 (range 7.6–26.1) kg, underwent the Fontan operation after having had the Norwood procedure with an RV-PA shunt. The patients were divided into 2 groups: left-sided RV-PA (L-RV-PA) (n = 129) with the shunt on the left and right-sided RV-PA (n = 32) with the shunt on the right side of the neoaorta. Angiographic data obtained before the Fontan and all cardiac catheterization interventions were analysed retrospectively. RESULTS Between the second and third stages, as well as directly before the Fontan operation, the L-RV-PA group required more PA catheter interventions (P = 0.001 and P = 0.03). In this group, the minimal left PA diameter was smaller than that in the R-RV-PA group (P = 0.021). Leaving the shunt open until the Fontan operation increased the rate of PA interventions in the L-RV-PA group (P = 0.001), but there is no evidence of the impact on the development of the left PAs (P = 0.075). There is also no evidence that the type of the second-stage procedure influences the intervention rate before the Fontan procedure (P = 0.14). CONCLUSIONS Children who have the L-RV-PA shunt require more PA catheter interventions. The right-sided RV-PA shunt and the subsequent Glenn anastomosis in the place of the shunt are associated with distortion-free and more symmetrical development of the central PAs.

scholarly journals Impact of Inspired Gas Mixtures on Preoperative Infants With Hypoplastic Left Heart Syndrome During Controlled Ventilation

Circulation ◽  
2001 ◽  
Vol 104 (suppl_1) ◽  
Author(s):  
Sarah Tabbutt ◽  
Chandra Ramamoorthy ◽  
Lisa M. Montenegro ◽  
Suzanne M. Durning ◽  
C. Dean Kurth ◽  
...  
Keyword(s):  
Oxygen Saturation ◽  
Hypoplastic Left Heart Syndrome ◽  
Oxygen Delivery ◽  
Superior Vena ◽  
Minute Ventilation ◽  
Recovery Period ◽  
Left Heart ◽  
Hypoplastic Left Heart ◽  
Left Heart Syndrome ◽  
The Impact

Background Management strategies for preoperative infants with hypoplastic left heart syndrome (HLHS) include increased inspired nitrogen (hypoxia) and increased inspired carbon dioxide (hypercarbia). There are no studies directly comparing these 2 therapies in humans. This study compares the impact of hypoxia versus hypercarbia on oxygen delivery, under conditions of fixed minute ventilation. Methods and Results Ten anesthetized and paralyzed preoperative infants with HLHS were evaluated in a prospective, randomized, crossover trial comparing hypoxia (17% F io 2 ) with hypercarbia (2.7% F ico 2 ). Each patient was treated in a random order (10 minutes per condition) with a recovery period (15 to 20 minutes) in room air. Arterial (Sa o 2 ) and superior vena caval (Sv o 2 ) co-oximetry and cerebral oxygen saturation (Sc o 2 ) measurements were made at the end of each condition and recovery period. Sc o 2 was measured by near infrared spectroscopy. Hypoxia significantly decreased both Sa o 2 (−5.2±1.1%, P =0.0014) and Sv o 2 (−5.6±1.7%, P =0.009) compared with baseline, but arteriovenous oxygen saturation (AV o 2 ) difference (Sa o 2 −Sv o 2 ) and Sc o 2 remained unchanged. Hypercarbia decreased Sa o 2 (−2.6±0.6%, P =0.002) compared with baseline but increased both Sc o 2 (9.6±1.8%, P =0.0001) and Sv o 2 (6±2.2%, P =0.022) and narrowed the AV o 2 difference (−8.5±2.3%, P =0.005). Both hypoxia and hypercarbia decreased the balance between pulmonary and systemic blood flow (Qp:Qs) compared with baseline. Conclusions In preoperative infants with HLHS, under conditions of anesthesia and paralysis, although Qp:Qs falls in both conditions, oxygen delivery is unchanged during hypoxia and increased during hypercarbia. These data cannot differentiate cerebral from systemic oxygen delivery.

Abstract 14882: Outcomes of Heart Failure in Hypoplastic Left Heart Syndrome and the Impact of Recovery

Circulation ◽  
2020 ◽  
Vol 142 (Suppl_3) ◽  
Author(s):  
Aine Lynch ◽  
Aamir Jeewa ◽  
Sunghoon Minn ◽  
Katelyn Arathoon ◽  
Jenna Ashkanase ◽  
...  
Keyword(s):  
Heart Failure ◽  
Hypoplastic Left Heart Syndrome ◽  
Age At Onset ◽  
Angiotensin Converting Enzyme Inhibition ◽  
Left Heart ◽  
Hypoplastic Left Heart ◽  
Similar Frequency ◽  
Free Survival ◽  
Left Heart Syndrome ◽  
The Impact

Introduction: Survival for children with hypoplastic left heart syndrome (HLHS) and right ventricular (RV) dysfunction (dysfxn) remain poor. We describe outcomes of HLHS patients with RV dysfxn and the long-term impact of interval normalization. Methods: The SickKids Heart Failure (HF) Database is a retrospective cohort of HF patients from 2001 to 2017. We included patients with HLHS and at least mild RV dysfxn on echocardiogram lasting >30 days, or death or transplant within 30 days of onset of dysfxn. We defined normalization as normal RV function (fxn) lasting > 30 days after dysfxn onset. The primary endpoint was death or transplant. Descriptive statistics and time to event analysis were used. Results: We identified 99 patients with a median (IQR) age at onset of dysfxn of 2.6 (1 - 5.3) months. Of these, 51 (52%) had normalization of RV fxn for a median (IQR) duration of 3.5 (0.8 - 9.1) years and 28 (55%) had multiple periods of normal fxn. Of those who normalized, 4 (8%) normalized within a few months post Glenn procedure and 6 (13%) had normal fxn at last follow up. Patients with and without normalization had similar frequency of severe dysfxn (41 vs 52%, p=0.6). Normalization of RV fxn correlated with improved transplant free survival (78 % vs. 14 %, p<0.001, Figure 1C) independent of surgical stage (p=0.03, figure 1D). Table 1 shows risk factors independently associated with transplant-free survival, including angiotensin converting enzyme inhibition. Conclusions: HLHS patients with RV dysfxn have poor transplant-free survival, especially those at interstage, but outcomes improve in those with persistent normalization of fxn. Factors predicting normalization need further study

The impact of morphological characteristics on late outcomes in patients born with hypoplastic left heart syndrome†

2019 ◽  
Vol 56 (3) ◽  
pp. 557-563 ◽  
Author(s):  
David P Newland ◽  
Chin L Poh ◽  
Diana Zannino ◽  
Patrick Elias ◽  
Christian P Brizard ◽  
...  
Keyword(s):  
Adverse Events ◽  
Hypoplastic Left Heart Syndrome ◽  
Fontan Procedure ◽  
Morphological Characteristics ◽  
Left Heart ◽  
Hypoplastic Left Heart ◽  
Late Morbidity ◽  
Fontan Failure ◽  
Left Heart Syndrome ◽  
The Impact

AbstractOBJECTIVESPatients with hypoplastic left heart syndrome are at high risk of late adverse events after Fontan completion, but it is unclear whether their morphological characteristics influence these outcomes.METHODSRetrospective review of the data from the Australian and New Zealand Fontan Registry identified 185 patients with hypoplastic left heart syndrome who survived to hospital discharge after Fontan completion. Their outcomes were reviewed to identify predictors of adverse events with a particular focus on the impact of morphological characteristics. All available echocardiographic parameters were collected, and the hypoplasia of the left ventricle was subjectively considered to be mild, moderate or severe.RESULTSThe mean follow-up after the Fontan procedure was 6.4 ± 4.7 years. The median age at Fontan procedure was 4.41 years, 95% (176/185) of patients underwent an extracardiac conduit Fontan procedure and 71% (132/185) of those were fenestrated. At 15 years after Fontan, freedom from death and cardiac transplantation was 90% [95% confidence interval (CI) 85–97], freedom from Fontan failure was 78% (95% CI 70–87) and freedom from adverse events was 32% (95% CI 22–46). Morphological parameters did not influence transplant-free survival or Fontan failure. Independent risk factors predicting higher incidence of adverse events included aortic atresia (P = 0.003).CONCLUSIONSThe long-term survival of Fontan survivors with hypoplastic left heart syndrome is excellent and appears comparable to that of the general Fontan population. However, intrinsic morphological characteristics may continue to burden patients with late morbidity.

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