OBJECTIVE: To report our experience of fetal aortic valvuloplasty (FAV) for critical aortic stenosis (AS), with a focus on the postnatal evolution of the patients.
METHODS: This was a retrospective study including all fetuses with critical AS which underwent FAV in a single center between January 2011 and June 2022. FAV was performed under ultrasound guidance. Technical success was based upon balloon inflation across the aortic valve and improvement of the antegrade aortic flow across the aortic valve. At birth, a biventricular circulation (BVC) strategy was decided assuming the left ventricular (LV) systolic and diastolic function would ensure the systemic circulation.
RESULTS: Sixty-three FAV procedures were performed in 58 fetuses, at a median (range) gestational age of 26.2 (20.3-32.2) weeks. The procedure was technically successful in 50/58 (86.2%) fetuses. There were 11/58 (19.0%) cases of in-utero demise and 9/58 (15.5%) terminations of pregnancy. No patient was liveborn after an unsuccessful procedure. Thirty-eight (65.5%) infants were liveborn, at a median (range) gestational age of 38.1 (29.0-40.6) weeks, of whom 21 (55.3%) required prostaglandin treatment. Twenty-eight of the 38 (73.7%) liveborn children (48.3% of the study population) entered the BVC pathway at birth. Among them, 20 (71.4%) required an aortic valvuloplasty procedure at birth (11 (55.0%) percutaneous balloon, nine (45.0%) surgical) and eight (28.6%) did not require any treatment at birth, but, of these, five (62.5%) underwent surgical valvuloplasty between day 26 and day 1200 of age. Eleven (39.3%) of the infants with BVC at birth required a second intervention and four (14.3%) of them required a third intervention. Two (7.1%) infants who entered the BVC pathway at birth underwent conversion to univentricular circulation (UVC). None of the surviving children with BVC developed pulmonary hypertension. The overall survival rate in those with BVC at birth was 22/28 (78.6%) at a median (range) follow-up of 23.3 (2.0-112.6) months. Ten of the 58 (17.2%) patients had UVC at birth. Among these, six (60.0%) received compassionate care from birth and four (40.0%) underwent surgery. Three of the 10 patients who had UVC at birth were still alive at the latest follow-up assessment, at a median (range) gestational age of 24.3 (8.3-48.7) months.
CONCLUSIONS: FAV for critical AS led to increase of antegrade aortic flow in 86.2% of fetuses, with BVC being achieved in 48.3% (73.7% of the liveborn cases). Among patients with BVC at birth, the rate of reintervention was high, but 78.6% of these children were alive at the latest evaluation. © 2024 The Authors. Ultrasound in Obstetrics & Gynecology published by John Wiley & Sons Ltd on behalf of International Society of Ultrasound in Obstetrics and Gynecology.

UNASSIGNED: Hybrid approach in hypoplastic left heart syndrome (HLHS) implies placement of bilateral pulmonary artery bands and ductal stenting. This approach serves as a less invasive surgical-interventional alternative to the Norwood procedure with reported equivalent survival. Potential benefits include deferrable surgical risks and in some borderline left ventricle patients, when combined with a restrictive inter-atrial communication, it offers a chance for biventricular circulation.
UNASSIGNED: Term newborn with total anomalous pulmonary venous connection (TAPVC), hypoplastic left heart, small aortic arch, and atrial septal defect (ASD) who was multi-disciplinary deemed eligible for a hybrid palliation performed on day six of life after TAPVC surgical correction. Subsequently, percutaneous ductal stent implantation was performed (two Sinus Superflex-DS stents) on day 11 of life. Magnetic resonance imaging (MRI) showed a hypoplastic non-apex-forming left ventricle (LV) [left ventricular end-diastolic volume index (LVEDVI) 15 mL/m2]. On day 27th of life, the ASD was closed surgically with a fenestrated patch. At 2 months of age, control cardiac MRI showed striking LV growth (LVEDVI 37 mL/m2). Herein an interventional hybrid take-down was performed with dilatation of both pulmonary arterial bands and closure of the ductal stent with an Amplatzer Vascular plug II, thus achieving biventricular circulation.
UNASSIGNED: Hybrid approach has proven as a safe alternative to the Norwood procedure for HLHS. This case illustrates the importance of multi-disciplinary care in high-risk two ventricular patients by means of LV rehabilitation. Further studies are needed to systematically define and understand the intricacies of potential candidates for staged biventricular repair initially managed with hybrid approach.

Critical aortic stenosis that appears in mid-gestation tends to develop to growth retardation of left ventricle, known as hypoplastic left heart syndrome (HLHS). Despite better clinical management of HLHS, the morbidity and mortality rates of univentricular circulation patients remain high. In this paper, we sought to perform a systematic review and meta-analysis to know the outcomes of fetal aortic valvuloplasty in critical aortic stenosis patients.
This systematic review and meta-analysis was conducted according to the Preferred Reporting Items for Systematic Review and Meta-Analyses (PRISMA) statement. A systematic search on fetal aortic valvuloplasty procedure for critical aortic stenosis was performed through PubMed, Scopus, EBSCOhost, ProQuest, and Google Scholar. The primary endpoint of each group was overall mortality. We used R software (version 4.1.3) to estimate the overall proportion of each outcome using random-effects model of proportional meta-analysis.
A total of 389 fetal subjects from 10 cohort studies were included in this systematic review and meta-analysis. Fetal aortic valvuloplasty (FAV) was successfully performed in 84% of patients. It revealed a successful conversion to biventricular circulation rate of 33% with a mortality rate of 20%. Bradycardia and pleural effusion requiring treatment were two most common fetal complications, whereas maternal complication reported was only placental abruption in one patient.
FAV has a high technical success rate with the ability to achieve biventricular circulation and a low rate of procedure-related mortality if carried out by experienced operators.

暂无摘要。

Despite significant improvements in the management of Fontan circulation in patients with single ventricle physiology, long-term outcomes continue to be suboptimal. Conversion to biventricular circulation is increasingly gaining popularity, particularly in the subset of patients who are not ideal Fontan candidates. Meticulous image-guided planning, extensive preoperative discussions, and a team-based approach are required for successful execution of complex biventricular conversion. A segmental approach to the anatomy of the heart defect allows methodical planning of the technique of biventricular conversion. Ventricular size and function continue to be the Achilles heel of successful biventricular repair. Long-term studies comparing outcomes in patients converted to biventricular circulation to those in patients with Fontan physiology are required to appropriately tailor management approaches to an individual patient.

OBJECTIVE: Fetal aortic valvuloplasty (FAV) may avoid progression of critical aortic stenosis (CAS) to hypoplastic left ventricle, improving the options for biventricular circulation (BVC). We describe the results of FAV in 2 referral centers in Spain.
METHODS: We analyzed all FAVs performed in the period 2007-2015. The selection of candidates, the technique, and postnatal management were made following an agreed protocol. A descriptive analysis of survival, type of circulation after birth, and complications was made, considering all deaths in the first 48 h after FAV as FAV-related.
RESULTS: FAV was performed in 28 fetuses at a median gestational age (GA) of 23 weeks (range, 20-32). FAV was technically successful in 22 (78.6%), of whom 11 were born alive and with intention to treat. Eight (72.7%) resulted in BVC and 3 (27.3%) in univentricular circulation. The rate of FAV-related deaths was 32%. These patients underwent FAV earlier than live-born fetuses (median GA at FAV 22 weeks [range, 20.0-25.0] vs. 24.5 weeks [range, 21.0-32.0], respectively, p = 0.031).
CONCLUSIONS: A significant proportion of fetuses with CAS who undergo technically successful FAV have BVC postnatally. However, FAV implies a high risk of fetal death, which highly depends on the GA at which this intervention is required.