Hypoplastic left heart syndrome with prenatally diagnosed foramen ovale restriction: diagnosis, management and outcome

Jadczak, Anna; Respondek-Liberska, Maria; Sokołowski, Łukasz; Chrzanowski, Jędrzej; Rizzo, Giuseppe; Júnior, Edward Araujo; Bravo-Valenzuela, Nathalie Jeanne; Axt-Fliedner, R; Słodki, Maciej

doi:10.1080/14767058.2020.1716717

Cited by 15 publications

(34 citation statements)

References 25 publications

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“…What is interesting, in our group FO restriction did not corresponded with higher mortality, what can be explained by the fact that it was not detected until 36 week of gestation. The study of Jadczak et al [35] showed that earlier development and longer presence of FO restriction is associated with higher short-term mortality.…”

Section: Discussionmentioning

confidence: 99%

Hypoplastic left heart syndrome: from the prenatal to the postnatal period

et al. 2021

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Objectives: To analyse a population of foetuses with prenatally diagnosed hypoplastic left heart syndrome (HLHS). Material and methods: Retrospective study of foetuses diagnosed with HLHS between 2013 and 2017 in a referral centre. Results: HLHS was found in 9.7% (65/665) of foetuses with cardiovascular abnormalities (CVA). As an isolated anomaly, HLHS was present in 40% of cases; in 24.5% other CVA were detected; in 14%, CVA and extracardiac anomalies; and in 21.5% only extracardiac malformations. Genetic disorders were present in 18.4% (12/65) of foetuses. 42% of cardiovascular and 25% of extracardiac anomalies were diagnosed postnatally. There were 10 (15.4%) elective terminations, 1 (1.5%) spontaneous foetal demise. Two newborns died after birth before surgery. Of the 52 children who underwent Norwood surgery, 13 (25%) died (9 with additional anomalies, and 4 with isolated HLHS). Of the 38 children who underwent stage II surgery, 2 (5.2%) with isolated HLHS died, and 1 (2.6%) with CVA. Conclusions: A diagnosis of HLHS is an indication for a detailed examination of cardiac and noncardiac structures. It is advisable to consider genetic testing, together with the microarray assessment. The prognosis depends on underlying cardiac and extracardiac anomalies and coexisting genetic defects.

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Section: Discussionmentioning

confidence: 99%

Hypoplastic left heart syndrome: from the prenatal to the postnatal period

et al. 2021

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“…Diagnosis of a highly restrictive or intact atrial septum (HR/IAS) in the context of a fetus with hypoplastic left heart syndrome 1 is managed differently across the world, with the divide not defined by resource availability alone. There are many case series [2][3][4][5][6][7] like this study by Generali and colleagues, 8 published in this issue of the World Journal. Small numbers of patients accrued over several decades, handled in different ways and likely by different key individuals in interventional cardiology and surgical roles.…”

mentioning

confidence: 82%

Invited Commentary: What Do We Achieve With Expedited Left Atrial Decompression?

Winlaw

Heydarian

Shahanavaz

2022

World J Pediatr Congenit Heart Surg

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“…Notably, HLH may be accompanied by different degrees of foramen ovale restriction (up to 22%), potentially leading to chronic left atrial hypertension, which is transmitted onto pulmonary venous structures, resulting in pulmonary hypertension and remodeling. Jadcak et al stated that an early appearance and a longer presence of FO obstruction rather than the extent of obstruction are associated with an increase in short‐term mortality 63 . Therefore, an additional thorough assessment of pulmonary venous (PV) Doppler (PVD) with a forward/reverse velocity‐time integral (VTI) ratio of <3 has been found to be the strongest predictor of postnatal hemodynamic stability and the need for fetal intervention 64 .…”

Section: Hypoplastic Left Heartmentioning

confidence: 99%

“…The aortic arch is hypoplastic, but the branching head vessels can be visualized (see Movie S3) extent of obstruction are associated with an increase in short-term mortality. 63 Therefore, an additional thorough assessment of pulmonary venous (PV) Doppler (PVD) with a forward/reverse velocity-time integral (VTI) ratio of <3 has been found to be the strongest predictor of postnatal hemodynamic stability and the need for fetal intervention. 64 Despite a trend toward improved preoperative morbidity in neonates with a prenatal diagnosis of HLH, a recent systematic review revealed no significant impact on preoperative or postoperative Stage I mortality.…”

Section: Hyp Opl a S Tic Lef T He Artmentioning

confidence: 99%

A “holistic” sonographic view on congenital heart disease: How automatic reconstruction using fetal intelligent navigation echocardiography eases unveiling of abnormal cardiac anatomy part II—Left heart anomalies

Weichert

2021

Echocardiography

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Volume ultrasound has been shown to provide valid complementary information on fetal anatomy. Four‐dimensional assessment (4D) of the fetal cardiovascular system using spatial–temporal image correlation (STIC) allows for detailed examination of a highly complex organ from the early second trimester onward. There is compelling evidence that this technique harbors quite a number of diagnostic opportunities, but manual navigation through STIC volume datasets is highly operator dependent. In fact, STIC is not incorporated yet into daily practice. Application of the novel fetal intelligent navigation echocardiography (FINE) considerably simplifies fetal cardiac volumetric examinations. This automatic technique applied on cardiac volume datasets reportedly has both high sensitivity and specificity for the detection of congenital heart defects (CHDs). Part I reviewed current data regarding detection rates of CHDs and illustrated the additional value of an automatic approach in delineating cardiac anatomy exemplified by congenital lesions of the right heart. In part II of this pictorial essay, we focused on left heart anomalies and aimed to tabulate recent findings on the quantification of normal and abnormal cardiac anatomy.

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Hypoplastic left heart syndrome with prenatally diagnosed foramen ovale restriction: diagnosis, management and outcome

Cited by 15 publications

References 25 publications

Hypoplastic left heart syndrome: from the prenatal to the postnatal period

Hypoplastic left heart syndrome: from the prenatal to the postnatal period

Invited Commentary: What Do We Achieve With Expedited Left Atrial Decompression?

A “holistic” sonographic view on congenital heart disease: How automatic reconstruction using fetal intelligent navigation echocardiography eases unveiling of abnormal cardiac anatomy part II—Left heart anomalies

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