Aortopulmonary Collateral Arteries in Noncyanotic Congenital Heart Disease.

Development of major aortopulmonary collateral arteries are strongly associated with cyanotic congenital heart disease. However, they have rarely been reported in noncyanotic congenital heart disease. We report a rare case of a newborn originally diagnosed with an atrial septal defect, a ventricular septal defect, and pulmonary arterial hypertension who underwent complete repair. Failure to progress postoperatively lead to the delayed diagnosis of aortopulmonary collateral arteries. Percutaneous embolization and surgical ligation of aortopulmonary collateral arteries resulted in rapid recovery.

Stent implantation of an unusual morphology patent ductus arteriosus via Glidesheath slender.

The procedure of stenting the patent ductus arteriosus (PDA) is a palliative procedure applied as an alternative to surgery in newborns with ductus-dependent pulmonary circulation. However, it is still a very challenging method in patients with aortic arch anomalies. We describe our experience with a newborn with right atrial isomerism and dextrocardia, complete atrioventricular septal defect, aortic outlet right ventricle with pulmonary atresia, right aortic arch, and a PDA from the left innominate artery. Because the PDA was long and tortuous, we preferred placing three short stents instead of a single long stent. The procedure applied the femoral artery approach with a Glidesheath Slender to decrease arterial injuries. PDA stenting in challenging morphologies can be performed successfully using multiple short stents and via Glidesheath Slenders.

Atresia bronquial / Bronchial atresia

Introducción: La atresia bronquial es una patología poco frecuente, generalmente asintomática e incidental en las imágenes del tórax. La aproximación diagnóstica de esta entidad se puede hacer por radiografía y broncoscopia, donde se identifican algunos aspectos claves en el diagnóstico diferencial. De acuerdo las manifestaciones clínicas y posibles complicaciones el tratamiento puede ser desde conservador hasta quirúrgico con resección lobar o segmentaria. Objetivo: Presentar las características de un caso con atresia bronquial. Presentación de caso: Se presenta un caso de atresia bronquial en una paciente de 19 años de edad cuyo tratamiento definitivo fue bilobectomía media-inferior derecha. Conclusiones: La atresia bronquial es una entidad infrecuente que puede cursar de manera asintomática y ser detectada por un hallazgo radiológico en pacientes adultos de manera incidental. El diagnóstico se puede confirmar por broncoscopia y el tratamiento casi siempre es quirúrgico(AU)

Introduction: Bronchial atresia is a rare disease, generally asymptomatic and incidental in chest images. The diagnostic approach of this entity can be done by radiography and bronchoscopy, some key aspects are identified in the differential diagnosis. According to the clinical manifestations and possible complications, the treatment can range from conservative to surgical with lobar or segmental resection. Objective: To describe a case of bronchial atresia. Case report: A case of bronchial atresia is reported in a 19-year-old patient whose definitive treatment was a right-lower-middle bilobectomy. Conclusions: Bronchial atresia is a rare entity that can occur asymptomatically and be detected incidentally by a radiological finding in adult patients. The diagnosis can be confirmed by bronchoscopy, and treatment is almost always surgical(AU)

Safe Decompression of the Right Ventricle for PAIVS in Neonates With Coronary Fistulae: Including the Selective Use of Fistula Ligation to Avoid Coronary Steal.

BACKGROUND: There are a number of surgical and interventional treatment options for infants with pulmonary atresia with intact ventricular septum (PAIVS). In our practice, we characterize coronary fistulae and interruptions with angiography in the newborn and have developed a strategy to safely decompress the right ventricle in association with ligation of fistulae if necessary. METHODS: All infants operated for PAIVS at age < 60 days from 1999 to 2018 were retrospectively studied. Pre- and postoperative variables were collected, angiograms were reviewed, and a territory score was created to grade the severity of coronary abnormalities. This study focused on the subgroup of patients who had early surgical decompression of the right ventricle. RESULTS: A total of 77 patients were included, with a mean follow-up of 8.6 years. Of these, 55 (71%) had coronary fistulae, including 28 (36%) with coronary artery interruption. Right ventricular decompression (RVD) was performed in 47 (60.5%) patients. There was no 30-day mortality in those who underwent RVD, whereas 6 (20%) without RVD died within 30 days (P = .003). Ten-year survival was 97.8% and 73.3% for RVD and non-RVD, respectively. In order to prevent coronary steal, 17 patients underwent coronary fistula ligation as their RV was decompressed with 100% early and late survival. CONCLUSION: Early and late survival in infants with PAIVS is better if the RV can be decompressed. Coronary fistula ligation with RVD has been introduced without an adverse outcome in selected patients with large fistulae.

Balloon Valvuloplasty via the Pulmonary Artery Trunk for Treating Neonates With Severe Pulmonary Valve Disease.

BACKGROUND: Percutaneous balloon pulmonary valvuloplasty has proven to be a standard of care for neonates with severe pulmonary valve disease. However, the peripheral vessel injury, tricuspid chordae tendineae rupture, and cardiac tamponade could occur. Recently, we performed balloon valvuloplasty through pulmonary artery trunk. To date, the obtained outcome was promising. METHODS: Between January 2018 and December 2018, three neonates with critical pulmonary stenosis and two with membranous pulmonary atresia with intact ventricular septum were enrolled in our center. Balloon valvuloplasty through pulmonary artery trunk was performed in all patients. A 2-cm parasternal incision was made in the left third intercostal space. A guidewire was used to advance or perforate the pulmonary valve from the pulmonary artery trunk into the right ventricle, followed by balloon dilation of the valve. RESULTS: The procedure was successful in all patients. The oxygen saturation increased immediately after the balloon dilation, while the right ventricular systolic pressure and the gradient across the pulmonary valve decreased. No severe complications occurred. CONCLUSIONS: Balloon valvuloplasty through the pulmonary artery trunk is a safe and feasible alternative procedure. Thus, it could serve as a supplementary choice for treating severe pulmonary valve disease.

Survival With Respect to Morphology in Pulmonary Atresia and Intact Ventricular Septum in Sweden.

BACKGROUND: Patients born with pulmonary atresia and intact ventricular septum represent a challenge to pediatric cardiologists. Our objective was to study changes in survival with respect to morphology in all children born with pulmonary atresia and intact ventricular septum in Sweden during 36 years. METHODS: A retrospective, descriptive study based on medical reports and echocardiographic examinations consisting of those born between 1980 and 1998 (early group) and those born between 1999 and 2016 (late group). RESULTS: The cohort consists of 171 patients (early group, n = 86 and late group, n = 85) yielding an incidence of 4.35 and 4.46 per 100,000 live births, respectively. One-year survival in the early group was 76% compared to 92% in the late group (P = .0004). For patients with membranous atresia, one-year survival increased from 78% to 98%, and for muscular pulmonary atresia, from 68% to 85%. In patients with muscular pulmonary atresia and ventriculocoronary arterial communications, there was no significant increase in survival. Risk factors for death were being born in the early time period hazard ratio (HR), 6; 95% CI (2.33-14.28) P = .0002, low birth weight HR, 1.26; 95% CI (1.14-1.4) P < .0001 and having muscular pulmonary atresia HR, 3.74; 95% CI (1.71-8.19) P = .0010. CONCLUSION: The incidence of pulmonary atresia and intact ventricular septum remained unchanged during the study period. Survival has improved, especially for patients with membranous pulmonary atresia, while being born with muscular pulmonary atresia is still a risk factor for death. To further improve survival, greater focus on patients with muscular pulmonary atresia and ventriculocoronary arterial communications is required.

Unusual Case of Common Arterial Trunk With Atresia of the Right Pulmonary Artery and Aortopulmonary Collaterals.

We describe a two-year-old African girl with late diagnosis of unusual case of common arterial trunk with two separate pulmonary artery branch origins from the ascending aorta, hypoplastic right pulmonary artery that becomes atretic and reconstitutes at hilum, and three aortopulmonary collaterals providing right lung blood supply. She underwent single-stage intracardiac repair and unifocalization of collaterals.

A case of COVID-19 in a patient with a univentricular heart post total cavopulmonary connection (Fontan) surgery.

Coronavirus disease 2019 (COVID-19) has caused a global pandemic which has affected patients and healthcare systems around the world. Patients with underlying health conditions seem to be more severely affected. There are limited reports of patients with univentricular circulations and COVID 19; thus, we report a case of COVID-19 in a patient with a univentricular circulation.

Transcatheter Approach for Critical Pulmonary Stenosis or Pulmonary Atresia with Intact Ventricular Septum in Young Infants Using the Simmons Catheter.

METHODS AND RESULTS: We retrospectively reviewed 52 young infants, 41 of whom had CPS and 11 had PA/IVS, in a single center from June 2009 to October 2017. Patients were divided into three groups according to the type of catheter used to enter through the RVOT. The unique structure of the Simmons catheter allowed it to be maneuvered directly into the RVOT within a few minutes. Compared with the other two groups, the Simmons catheter group had a significantly shorter fluoroscopy time entering through the RVOT (P < 0.001) and a shorter total X-ray exposure time (P < 0.001). Furthermore, compared with the floating catheter group, the success rate of surgery was much higher in the Simmons catheter group (P < 0.001). CONCLUSIONS: The Simmons catheter is a safe and effective method to enter through the RVOT in infants with CPS or PA/IVS. Therefore, the Simmons catheter could be an alternative catheter when entering through the RVOT in young infants, especially neonates with low birth weight.

Ventricular Septal Defect Pulmonary Atresia: Provisional Bifurcation Strategy for Confluence Stenosis During Patent Ductus Arteriosis Stenting.

Neonates with pulmonary atresia survive only if duct patency is maintained before staged surgical repair. Ductal stenting is an effective alternative to conventional shunt surgery, as it avoids thoracotomy. With today's generation of coronary stents having better profile, flexibility, and trackability, it may be achieved safely, with less difficulty than previously described. The strategy during patent ductus arteriosus (PDA) stenting with trifurcation narrowing is a real challenge. We present an underweight newborn with ventricular septal defect pulmonary atresia, restrictive PDA, and bilateral branch stenosis at the ostia. We performed a successful PDA stenting as well as addressed the ostia of branch pulmonary arteries.

Tetralogy of Fallot With Absent Pulmonary Valve and Nonconfluent Pulmonary Arteries: A Management Conundrum.

Tetralogy of Fallot with absent pulmonary valve syndrome is a rare form of congenital heart disease. Among the different variations with this rare anomaly is nonconfluent pulmonary artery branches with anomalous origin of the left pulmonary artery from the ductus arteriosus. The authors present one such case which was diagnosed prenatally to have tetralogy of Fallot with absent pulmonary valve and identified postnatally to have nonconfluent pulmonary artery branches in addition. We discuss the conundrum of respiratory management in this patient pre- and postoperatively due to a unique ventilation perfusion mismatch problem, which varies between the two lungs.

Right Ventricle-Dependent Coronary Circulation: Location of Obstruction Is Associated With Survival.

BACKGROUND: Pulmonary atresia with intact ventricular septum (PAIVS) with right ventricle-dependent coronary circulation (RVDCC) carries suboptimal outcomes primarily due to cardiac ischemia. We hypothesize clinical outcomes are affected by the level of coronary obstruction, a surrogate for vulnerable myocardium. METHODS: We conducted a single-institution retrospective analysis of all neonates with PAIVS with RVDCC from 1995 to 2017. RVDCC was defined as the presence of any coronary-cameral fistula with coronary obstruction proximal to the fistula and angiographic evidence of RV perfusion of the myocardium through the fistulous communication. Location of coronary obstruction was categorized as either proximal or distal segments, using the SYNTAX score criteria. Transplant-free survival was compared between patients with proximal and distal obstruction, then these groups were compared with patients without RVDCC. RESULTS: Of 103 neonates with PAIVS, 28 (27%) had RVDCC: 18 proximal (64%), 10 distal (36%). Median age at last follow-up for patients with RVDCC was 1.8 years (interquartile range, 0.3-8.1 years). All deaths (10 of 28, 36%) occurred at 6 months old or earlier. Proximal coronary artery obstruction was associated with decreased transplant-free survival relative to distal obstruction (hazard ratio = 3.63; 95% confidence interval, 1.01-13.00; P = .048). Transplant-free survival at 1 year was 33% and 70% in the proximal and distal obstruction groups, respectively. Compared with patients without RVDCC, patients with proximal obstruction had significantly lower transplant-free survival (P < .001), whereas patients with distal obstruction did not (P = .217). CONCLUSIONS: The location of coronary artery obstruction affects clinical outcome and may represent a potential branch point in the management for PAIVS with RVDCC.

Pulmonary Atresia with Ventricular Septal Defect: Rare Presentation with Coronary-to-Pulmonary Artery Collaterals from Both Right and Left Coronaries.

Pulmonary atresia with ventricular septal defect and coronary-dependent pulmonary circulation arising from both major coronary arteries is rare. Dependence of pulmonary blood flow on the coronaries and the risk of early development of pulmonary vascular obstructive disease warrant early surgical repair in these patients. We report a case of a ten-month-old infant with pulmonary atresia with ventricular septal defect and coronary artery-to-main pulmonary artery connections who was successfully managed with ligation of the coronary fistulas and intracardiac repair.

Aortopulmonary Window With Pulmonary Atresia and Interrupted Aortic Arch: A Very Rare Triad.

Aortopulmonary window (APW) is a rare lesion, accounting for 0.2% to 0.6% of all congenital heart diseases. We report a rare case of an infant with APW, interrupted aortic arch, and pulmonary atresia with intact interventricular septum and right ventricle-dependent coronary circulation. This report describes the anatomy of this lesion set, the complex surgical palliation that was required, and the management of postoperative complications.

Perforation of the atretic pulmonary valve using chronic total occlusion (CTO) wire and coronary microcatheter.

BACKGROUND AND OBJECTIVE: Chronic total occlusion (CTO) guidewire have been recently reported as an alternative to radiofrequency for perforating atretic pulmonary valve. Since procedure failures or perforation of the right ventricle still occurred with CTO, we tried to enhance the stability, steering, and pushability of the wire using a microcatheter in order to improve the safety and efficacy of the procedure. METHODS: We performed pulmonary valve perforation with CTO guidewire and microcatheter in five consecutive newborns with pulmonary atresia with intact ventricular septum (PA-IVS) under fluoroscopic and echocardiographic control. RESULTS: The valve was easily perforated at the first attempt for all patients. After perforation, the microcatheter positioned in the main pulmonary artery allowed the exchange of the CTO guidewire for a more flexible wire, avoiding lesion and facilitating manipulation in the distal pulmonary branch arteries. The pulmonary valve was then dilated with balloons of increasing size as usually performed. We did not experience any procedural or early complications. Blalock-Taussig shunt was performed in 2 children because of a persistent cyanosis, 4 and 10 days after perforation. CONCLUSIONS: The combined use of a CTO guide and a microcatheter appears to be a safe and reliable technique for perforating the pulmonary valve of newborns with PA-IVS. Further procedures with this approach are needed to confirm this first experience.

The outcome of adults born with pulmonary atresia: High morbidity and mortality irrespective of repair.

OBJECTIVES: To describe the characteristics and long-term outcome of a large adult cohort with pulmonary atresia. BACKGROUND: Patients with pulmonary atresia (PA) are a heterogeneous population in terms of anatomy, physiology and surgical history, and their management during adulthood remains challenging. METHODS: Data on all patients with PA followed in our center between January 2000 and March 2015 were recorded. Patients were classified into the following groups: PA with ventricular septal defect (PA-VSD, 1), PA with intact ventricular septum (PA-IVS, 2) and other miscellaneous PA (PA-other, 3). RESULTS: Two-hundred twenty-seven patients with PA were identified, 66.1% female, mean age 25.5 ±â€¯8.7 years. Over a median follow-up of 8.8 years, 49 (21.6%) patients had died: heart failure (n = 21, 42.8%) and sudden cardiac death (n = 8, 16.3%) were the main causes. There was no significant difference in mortality between the 3 Groups (p = 0.12) or between repaired and unrepaired patients in Group 1 (p = 0.16). Systemic ventricular dysfunction and resting oxygen saturations were the strongest predictors of mortality. Additionally, 116 (51%) patients were hospitalized, driven mainly by the need for invasive procedures, heart failure and arrhythmias. CONCLUSIONS: Adult survivors with pulmonary atresia have a high morbidity and mortality irrespective of underlying cardiac anatomy and previous reparative or palliative surgery. We present herewith predictors of outcome in adult life that may assist with their tertiary adult congenital care.

Airway Compression After Unifocalization in Pulmonary Atresia and Aortopulmonary Collateral Arteries.

BACKGROUND: We hypothesized that reconstructed pulmonary artery (PA) size and postrepair PA pressure are associated with airway compression (AC) after complete unifocalization for pulmonary atresia, ventricular septal defects, and major aortopulmonary collateral arteries. METHODS: Complete unifocalization was performed in 48 consecutive patients between 2000 and 2016. Clinical course and outcome were reviewed, predictors for AC were identified by logistic regression, and the freedom from death was analyzed using Kaplan-Meier method. RESULTS: Postoperative respiratory distress occurred in 23 patients (48%), and AC occurred in 14 (29%). The median duration of follow-up was 3.7 years. AC was caused by central PA and aorta in 7, conduit in 3, and branch PA in 4. Surgical treatment was required in 5 patients (conduit downsizing, suspension of branch PA, conduit + aorta, branch PA + aorta, and aorta + trachea in 1 patient each). Three patients (21%) subsequently required airway stenting. Most (85.7%) of the AC occurred in patients with high right ventricular systolic pressure/left ventricular systolic pressure (>65%), large Nakata index (>200 mm2/m2), and large conduit index (>35 mm/m2). Patients with AC had significantly worse 3-year survival (no AC, 91.2%; AC, 64.2%; p = 0.01). Multivariate analysis identified higher right ventricular systolic pressure/left ventricular systolic pressure (p = 0.04), larger conduit index (p = 0.03), and Nakata index (p = 0.004) as predictors for AC. CONCLUSIONS: AC is a common cause of postoperative respiratory distress and tends to be associated with higher postrepair PA pressure, more frequent right ventricular dysfunction, and worse medium-term survival. The study underscores the importance of incorporating all available lung segments to achieve a low PA pressure, potentially preventing pathologic dilatation of the reconstructed PA. Management of patients with poor major aortopulmonary collateral arteries anatomy and physiology remains a challenge.

Postoperative feeding problems in patients with tetralogy of Fallot, pulmonary atresia, and major aortopulmonary collaterals undergoing unifocalisation surgery.

BACKGROUND: Patients with tetralogy of Fallot, pulmonary atresia, and major aortopulmonary collaterals are at risk for prolonged hospitalisation after unifocalisation. Feeding problems after congenital heart surgery are associated with longer hospital stay. We sought to determine the impact of baseline, intra-operative, and postoperative factors on the need for feeding tube use at the time of discharge. METHODS: We included patients with the aforementioned diagnosis undergoing unifocalisation from ages 3 months to 4 years from 2010 to 2016. We excluded patients with a pre-existing feeding tube. Patients discharged with an enteric tube were included in the feeding tube group. We compared the feeding tube group with the non-feeding-tube group by univariable and multi-variable logistic regression. RESULTS: Of the 56 patients studied, 41% used tube feeding. Median age and weight z-score were similar in the two groups. A chromosome 22q11 deletion was associated with the need for a feeding tube (22q11 deletion in 39% versus 15%, p=0.05). Median cardiopulmonary bypass time in the feeding tube group was longer (335 versus 244 minutes, p=0.04). Prolonged duration of mechanical ventilation was associated with feeding tube use (48 versus 3%, p=0.001). On multi-variable analysis, prolonged mechanical ventilation was associated with feeding tube use (odds ratio 10.2, 95% confidence intervals 1.6; 63.8). CONCLUSION: Among patients with tetralogy of Fallot, pulmonary atresia, and major aortopulmonary collaterals who were feeding by mouth before surgery, prolonged mechanical ventilation after unifocalisation surgery was associated with feeding tube use at discharge. Anticipation of feeding problems in this population and earlier feeding tube placement may reduce hospital length of stay.

Pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals: collateral vessel disease burden and unifocalisation strategies.

IntroductionThe optimal approach to unifocalisation in pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries (pulmonary artery/ventricular septal defect/major aortopulmonary collaterals) remains controversial. Moreover, the impact of collateral vessel disease burden on surgical decision-making and late outcomes remains poorly defined. We investigated our centre's experience in the surgical management of pulmonary artery/ventricular septal defect/major aortopulmonary collaterals.Materials and methodsBetween 1996 and 2015, 84 consecutive patients with pulmonary artery/ventricular septal defect/major aortopulmonary collaterals underwent unifocalisation. In all, 41 patients received single-stage unifocalisation (Group 1) and 43 patients underwent multi-stage repair (Group 2). Preoperative collateral vessel anatomy, branch pulmonary artery reinterventions, ventricular septal defect status, and late right ventricle/left ventricle pressure ratio were evaluated. RESULTS: Median follow-up was 4.8 compared with 5.7 years for Groups 1 and 2, respectively, p = 0.65. Median number of major aortopulmonary collaterals/patient was 3, ranging from 1 to 8, in Group 1 compared with 4, ranging from 1 to 8, in Group 2, p = 0.09. Group 2 had a higher number of lobar/segmental stenoses within collateral vessels (p = 0.02). Group 1 had fewer catheter-based branch pulmonary artery reinterventions, with 5 (inter-quartile range from 1 to 7) per patient, compared with 9 (inter-quartile range from 4 to 14) in Group 2, p = 0.009. Among patients who achieved ventricular septal defect closure, median right ventricle/left ventricle pressure was 0.48 in Group 1 compared with 0.78 in Group 2, p = 0.03. Overall mortality was 6 (17%) in Group 1 compared with 9 (21%) in Group 2.DiscussionSingle-stage unifocalisation is a promising repair strategy in select patients, achieving low rates of reintervention for branch pulmonary artery restenosis and excellent mid-term haemodynamic outcomes. However, specific anatomic substrates of pulmonary artery/ventricular septal defect/major aortopulmonary collaterals may be better suited to multi-stage repair. Preoperative evaluation of collateral vessel calibre and function may help inform more patient-specific surgical management.

[Prenatal interventional therapy in two cases with critical pulmonary stenosis or pulmonary atresia with intact ventricular septum].

Objectives: Two cases who underwent fetal pulmonary valvuloplasty (FPV) for pulmonary atresia with intact ventricular septum (PA-IVS) or critical pulmonary stenosis with intact ventricular septum (CPS-IVS) successfully were reported. The aim of the report was to explore the criteria for case selection, the technical essentials of FPV, and the postpartum outcome of the fetus. Methods: One case with PA-IVS and the other with CPS-IVS were enrolled in September 2016 and February 2017 in Guangdong General Hospital, and both cases were diagnosed with severe right ventricular dysplasia and tricuspid regurgitation by fetal echocardiogram. Parameters of right ventricle development and hemodynamics from echocardiography included tricuspid/mitral annulus (TV/MV), right ventricle/left ventricle long-axis (RV/LV), pulmonary/aortic annulus (PV/AV), tricuspid inflow duration/cardiac cycle, degree of tricuspid regurgitation (TR), blood flow direction of arterial duct and ductus venosus. Multidisciplinary team including the maternal-fetal cardiology, pediatric cardiology, cardiac surgery, obstetrics, neonatology and anesthesiology was summoned to discuss the indications and timing of PFV. Two cases underwent ultrasound-guiding trans-abdominal PFV at the 28 weeks of gestational age. Echocardiography was performed to observe the opening and closing of the pulmonary valve, and to evaluate the development of right ventricle and improvement in hemodynamics every 2-4 weeks until delivery. Results: From the technical perspective, pulmonary balloon valvuloplasty was successfully performed in these two cases. The opening of pulmonary valve improved in these two cases at 2-4 weeks after FPV. However, an obvious restenosis was detected in the first case at 5-8 weeks after FPV. In the first case, the echocardiography parameters including TV/MV, RV/LV, PV/AV and tricuspid inflow duration/cardiac cycle increased from 0.56, 0.42, 0.85,0.26 to 0.59, 0.51, 0.87, 0.32 at 5-8 weeks after FPV, respectively. However, the direction of blood flow through the arterial duct was still reverse. In the second case, TV/MV, RV/LV, PV/AV and tricuspid inflow duration/cardiac cycle ratio increased from 0.70, 0.63, 0.91,0.35 to 0.80, 0.80, 0.97, 0.42 at 5-8 weeks after FPV, respectively. The direction of blood flow through the arterial duct changed to bidirectional. Both fetuses were born alive. The first case underwent pulmonary valve commissurotomy and modified Blalock-Taussig shunt on the 8(th) day after delivery and received follow-up for 6 months. The strategy for the next-step therapy was still pending. The second case underwent transcutaneous pulmonary balloon valvuloplasty on the 19(th) day after delivery and received follow-up for 3 months. The opening of pulmonary valve improved obviously and the cardiac function was normal in the second case. Conclusions: FPV is safe and effective for fetus during the second and third trimester of pregnancy, and FPV is beneficial for the development of fetal ventricle, valve and large artery. In addition, FPV may help to avoid the postnatal surgery for isolated single ventricle, improve fetal heart failure and prevent fetal death.