A multidisciplinary planned approach is critical to successfully manage hypoplastic left heart syndrome with an intact atrial septum.
Over the past few decades, major advances in the form of surgical technique, catheter-based interventions, and critical care management have resulted in improved outcomes with hypoplastic left heart syndrome (HLHS) palliation. Despite this progress, the subset of HLHS that presents with a highly restrictive atrial septum (RAS) or intact atrial septum (IAS) continues to present a challenge. Both an IAS and RAS will produce left atrial and pulmonary venous hypertension. This is thought to lead to abnormal lung development, with reduction in lung perfusion characterized by congenital pulmonary lymphangiectasis and muscularization of pulmonary veins. This has been noted to occur as early as 23 weeks of gestation with histologic changes such as hypercapillarization and widened inter-airspace mesenchyme noted at fetal autopsy.
1Whether these changes are reversible with left atrial decompression, either prenatally by fetal intervention or postnatally, is unknown. Currently, the 1-year survival in the HLHS population with RAS/IAS is low, with survival between 35% and 50% reported in recent case series.
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- Lunkenheimer J.M.
- Abedini M.
- Herberg U.
- Berg C.
- Gembruch U.
- et al.
Left ventricular obstruction with restrictive inter-atrial communication leads to retardation in fetal lung maturation.
Prenat Diagn. 2015; 35: 463-470
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- Drogosz M.
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- Sleeper L.A.
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- Allan C.
- et al.
Echocardiographic predictors of neonatal illness severity in fetuses with critical left heart obstruction with intact or restrictive atrial septum.
Prenat Diagn. 2018; 38: 788-794
- Tanem J.
- Rudd N.
- Rauscher J.
- Scott A.
- Frommelt M.A.
- Hill G.D.
Survival after Norwood procedure in high-risk patients.
Ann Thorac Surg. 2020; 109: 828-833
In this issue of the JTCVS Open, Sood and colleagues
4present their institutional strategy for the management of HLHS/IAS. Their multidisciplinary approach involves cesarean delivery, airway and line establishment, and transfer to the cardiac catheterization suite within 30 minutes of delivery. Sternotomy and per-atrial balloon septostomy or atrial septal stenting is performed, followed by bilateral pulmonary artery bands and, in the majority of cases, a ductal stent (HS1P). The authors are to be congratulated on organizing a large group of experts and developing a reproducible approach to this critically ill, high-risk group of neonates. The management plan emphasizes, importantly, efficiency with times of 30 minutes from delivery to the catheterization suite and 5 minutes to per-atrial access. The outcomes on 15 neonates with HLHS/IAS briefly presented are notable, with 10 being discharged home after HS1P and 7 undergoing successful Norwood.
- Sood V.
- Zampi J.D.
- Romano J.C.
Hypoplastic left heart syndrome with an intact atrial septum.
J Thorac Cardiovasc Surg Open. 2020; 1: 51-56
Although the authors present a comprehensive plan, there are a few particulars that would be helpful in providing the complete picture. With regard to the atrial septal intervention, details regarding time to adequate septostomy, failure rate, and indication for surgical conversion would be helpful in understanding the limitations of this strategy. In addition, information about the reasons for mortality and failing to progress to the next stage may provide insight into the challenges these neonates may face secondary to pulmonary venous disease or unfavorable anatomy. We suspect that these specifics will be clarified in a future publication reporting on the outcomes of their strategy.
In closing, we are in full agreement with the authors in the need for institutions to have a multidisciplinary strategy to optimize the outcomes of high-risk neonates with HLHS/IAS. Our strategy has been to have elective cesarean delivery within the women’s center inside the children’s hospital. The newborn is resuscitated, prostaglandins are initiated, and the patient is transported immediately to the cardiac operating room, where the surgical team is standing by. Cardiac anesthesia intubates and places venous and arterial lines expeditiously. A surgical atrial septectomy is done followed by bilateral pulmonary banding. We favor tight bilateral pulmonary artery banding (1.8-2.2 mm), using supplemental oxygen if needed, as even mild pulmonary overcirculation is poorly tolerated in these patients.
- Barker G.M.
- Forbess J.M.
- Guleserian K.J.
- Nugent A.W.
Optimization of preoperative status in hypoplastic left heart syndrome with intact atrial septum by left atrial decompression and bilateral pulmonary artery bands.
Pediatr Cardiol. 2014; 35: 479-484
6We have kept these patients on prostaglandins for ductal patency and delayed the Norwood operation for at least 4 to 6 weeks to achieve a period of lung recovery. This period is geared toward nutrition, lowering respiratory support, and optimizing pulmonary function with diuresis and dual pulmonary hypertensive medications. This strategy has been used in 4 neonates with HLHS/IAS, with all undergoing Norwood followed by bidirectional cavopulmonary shunt (1 mortality after Glenn). How these high-risk babies will fare long term remains to be seen, but developing an institutional plan is undoubtedly the first step toward success.
- He D.
- Sinha P.
- Jonas R.A.
Pulmonary overcirculation following left atrial decompression in hypoplastic left heart syndrome with intact/restrictive atrial septum.
J Card Surg. 2012; 27: 758-759
- Left ventricular obstruction with restrictive inter-atrial communication leads to retardation in fetal lung maturation.Prenat Diagn. 2015; 35: 463-470
- Echocardiographic predictors of neonatal illness severity in fetuses with critical left heart obstruction with intact or restrictive atrial septum.Prenat Diagn. 2018; 38: 788-794
- Survival after Norwood procedure in high-risk patients.Ann Thorac Surg. 2020; 109: 828-833
- Hypoplastic left heart syndrome with an intact atrial septum.J Thorac Cardiovasc Surg Open. 2020; 1: 51-56
- Optimization of preoperative status in hypoplastic left heart syndrome with intact atrial septum by left atrial decompression and bilateral pulmonary artery bands.Pediatr Cardiol. 2014; 35: 479-484
- Pulmonary overcirculation following left atrial decompression in hypoplastic left heart syndrome with intact/restrictive atrial septum.J Card Surg. 2012; 27: 758-759
Published online: April 02, 2020
Accepted: March 20, 2020
Received in revised form: March 13, 2020
Received: March 13, 2020
Disclosures: The authors reported no conflicts of interest.
The Journal policy requires editors and reviewers to disclose conflicts of interest and to decline handling or reviewing manuscripts for which they may have a conflict of interest. The editors and reviewers of this article have no conflicts of interest.
© 2020 by The Authors. Published by Elsevier Inc. on behalf of The American Association for Thoracic Surgery.
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- Hypoplastic left heart syndrome with an intact atrial septumJTCVS OpenVol. 1
- PreviewFeature Editor's Note—Newborns with hypoplastic left ventricle and intact atrial septum are among the most challenging subgroups of patients. Despite the fact that most patients are now diagnosed antenatally by fetal echocardiography, the mortality in these patients remains high and the long-term prognosis is dismal. Herein, Dr Romano and colleagues present a team-based hybrid approach developed by the University of Michigan multidisciplinary group. One can only hope that this admirable, labor-intense collaborative effort by fetal and interventional cardiologists, pediatric cardiothoracic surgeons, pediatric intensivists, and palliative care and social work specialists will overcome the severity of the underlying condition and improve the outcomes of these patients.