Valvuloplasty in 103 fetuses with critical aortic stenosis: outcome and new predictors for postnatal circulation.

OBJECTIVES: To review our experience with fetal aortic valvuloplasty (FAV) in fetuses with critical aortic stenosis (CAS) and evolving hypoplastic left heart syndrome (eHLHS), including short- and medium-term postnatal outcome, and to refine selection criteria for FAV by identifying preprocedural predictors of biventricular (BV) outcome. METHODS: This was a retrospective review of all fetuses with CAS and eHLHS undergoing FAV at our center between December 2001 and September 2020. Echocardiograms and patient charts were analyzed for pre-FAV ventricular and valvular dimensions and hemodynamics and for postnatal procedures and outcomes. The primary endpoints were type of circulation 28 days after birth and at 1 year of age. Classification and regression-tree analysis was performed to investigate the predictive capacity of pre-FAV parameters for BV circulation at 1 year of age. RESULTS: During the study period, 103 fetuses underwent 125 FAVs at our center, of which 87.4% had a technically successful procedure. Technical success per fetus was higher in the more recent period (from 2014) than in the earlier period (96.2% (51/53) vs 78.0% (39/50); P = 0.0068). Eighty fetuses were liveborn after successful intervention and received further treatment. BV outcome at 1 year of age was achieved in 55% of liveborn patients in our cohort after successful FAV, which is significantly higher than the BV-outcome rate (23.7%) in a previously published natural history cohort fulfilling the same criteria for eHLHS (P = 0.0015). Decision-tree analysis based on the ratio of right to left ventricular (RV/LV) length combined with LV pressure (mitral valve regurgitation maximum velocity (MR-Vmax)) had a sensitivity of 96.97% and a specificity of 94.44% for predicting BV outcome without signs of pulmonary arterial hypertension at 1 year of age. The highest probability for a BV outcome was reached for fetuses with a pre-FAV RV/LV length ratio of < 1.094 (96.4%) and for those fetuses with a RV/LV length ratio ≥ 1.094 to < 1.135 combined with a MR-Vmax of ≥ 3.14 m/s (100%). CONCLUSIONS: FAV could be performed with high success rates and an acceptable risk with improving results after a learning curve. Pre-FAV RV/LV length ratio combined with LV pressure estimates were able to predict a successful BV outcome at 1 year of age with high sensitivity and specificity. © 2022 The Authors. Ultrasound in Obstetrics & Gynecology published by John Wiley & Sons Ltd on behalf of International Society of Ultrasound in Obstetrics and Gynecology.

Immediate effects and outcome of in-utero pulmonary valvuloplasty in fetuses with pulmonary atresia with intact ventricular septum or critical pulmonary stenosis.

OBJECTIVE: To assess the immediate effects of fetal pulmonary valvuloplasty on right ventricular (RV) size and function as well as in-utero RV growth and postnatal outcome. METHODS: Patients with pulmonary atresia with intact ventricular septum (PAIVS) or critical pulmonary stenosis (CPS) who underwent fetal pulmonary valvuloplasty at our center between October 2000 and July 2017 were included. Echocardiographic data obtained before and after the procedure were analyzed retrospectively (median interval after intervention, 1 (range, 1-3) days) for ventricular and valvular dimensions and ratios, RV filling time (duration of tricuspid valve (TV) inflow/cardiac cycle length), TV velocity time integral (TV-VTI) × heart rate (HR) and tricuspid regurgitation (TR) velocity. Longitudinal data were collected from only those fetuses followed up in our center. Outcome was assessed using the scoring system as described by Roman et al. for non-biventricular outcome. RESULTS: Thirty-five pulmonary valvuloplasties were performed in our institution on 23 fetuses with PAIVS (n = 15) or CPS (n = 8). Median gestational age at intervention was 28 + 4 (range, 23 + 6 to 32 + 1) weeks. No fetal death occurred. Immediately after successful intervention, RV/left ventricular length (RV/LV) ratio (P ≤ 0.0001), TV/mitral valve annular diameter (TV/MV) ratio (P ≤ 0.001), RV filling time (P ≤ 0.00001) and TV-VTI × HR (P ≤ 0.001) increased significantly and TR velocity (P ≤ 0.001) decreased significantly. In fetuses followed longitudinally to delivery (n = 5), RV/LV and TV/MV ratios improved further or remained constant until birth. Fetuses with unsuccessful intervention (n = 2) became univentricular, all others had either a biventricular (n = 15), one-and-a-half ventricular (n = 3) or still undetermined (n = 3) outcome. Five of nine fetuses with a predicted non-biventricular outcome, in which the procedure was successful, became biventricular, while two of nine had an undetermined circulation. CONCLUSION: In selected fetuses with PAIVS or CPS, in-utero pulmonary valvuloplasty led immediately to larger RV caused by reduced afterload and increased filling, thus improving the likelihood of biventricular outcome even in fetuses with a predicted non-biventricular circulation. © 2018 The Authors. Ultrasound in Obstetrics & Gynecology published by John Wiley & Sons Ltd on behalf of the International Society of Ultrasound in Obstetrics and Gynecology.

Maternal aspects of fetal cardiac intervention.

OBJECTIVES: Fetal cardiac interventions have the potential to alter natural disease progression and reduce morbidity and mortality in children. Although there are already encouraging data on fetal outcome, information on maternal morbidity and mortality after intervention is scarce. The aim of the present study was to assess maternal aspects, pregnancy-associated risks and adverse events in 53 intrauterine cardiac interventions. METHODS: Between October 2000 and December 2012, 53 fetal cardiac interventions were performed in 47 patients (43 aortic valve dilations in 39 patients, seven pulmonary valve dilations in six patients and three balloon atrioseptostomies in two patients). Median gestational age was 26 + 4 (range, 20 + 3 to 33 + 1) weeks. Interventions were performed by an ultrasound-guided percutaneous approach under general anesthesia. All medical records and patient charts were analyzed retrospectively. RESULTS: All women were considered to be healthy in the preoperative assessment; 39 (83%) patients continued pregnancy until term and eight of 47 patients had an intrauterine fetal death (IUFD) and were induced. Postoperative nausea was reported in 29.8% of patients and abdominal pain in 36.2% of patients on the day of surgery. Preterm contractions were observed in two patients; no preterm prelabor rupture of membranes occurred. One severe postpartum hemorrhage was observed in a patient with IUFD and subsequent induction; however, this was unrelated to the balloon valvuloplasty. No intensive care unit admission and no major anesthesia-associated complications (aspiration, anaphylactic reaction, cardiovascular collapse, damage to teeth, laryngeal damage, awareness or hypoxic brain damage) were observed. Maternal mortality was zero. A significant learning curve was observed in terms of duration of intervention. CONCLUSION: In our experience, percutaneous needle-guided fetal cardiac intervention seems to be a safe procedure for the mother. In 53 procedures no major maternal complication directly related to the intervention was observed.

Necrotizing enterocolitis after open cardiac surgery for congenital heart defects--a serious threat.

Neonates with congenital heart disease are at risk to develop necrotizing enterocolitis (NEC). Especially in the postoperative period after correction of a congenital heart defect this intestinal inflammatory disease can be a potentially life-threatening complication. The purpose of this case series is to define the specific features of NEC in the postoperative situation, with emphasis on diagnostic difficulties.Medical records of neonates developing severe NEC in the postoperative period after surgical correction of a congenital heart defect from 2008 to 2011 were reviewed and compared.7 neonates with NEC after surgical correction of a congenital heart defect were identified. In all 7 patients typical radiologic findings of NEC were missing. Diagnosis was late in all 7 cases; 5 patients already had intestinal perforation. In 3 patients NEC successfully managed before surgical correction of the congenital heart defect dramatically relapsed in the postoperative period. 2 patients showed intestinal mucormycosis, an entity not clearly differentiated from NEC. Due to progressed disease at diagnosis all patients required extensive surgical procedures; 2 of them subsequently died.In the postoperative situation after surgical correction of a congenital heart defect diagnosis of NEC might be challenging. The general condition of these children complicates clinical examination and characteristic radiologic signs are absent. Late diagnosis and exclusive pathogens cause high morbidity and mortality.

[Hepatic artery pseudoaneurysm following blunt abdominal injury]. / Arterielles Pseudoaneurysma der Leber nach stumpfem Bauchtrauma.

Posttraumatic hepatic artery pseudoaneurysms are a rare but life-threatening complication of blunt abdominal trauma with liver damage. We report the case of a child who developed a pseudoaneurysm of the right hepatic artery after a bicycle accident with central liver rupture. After an episode of hemodynamically relevant hemobilia due to delayed bleeding, the asymptomatic pseudoaneurysm was diagnosed coincidentally by ultrasound. Because of the progression in size angiographic coiling was performed and led to thrombotic occlusion of the pseudoaneurysm. After a symptom-free period of 1 month the child required surgery because of acute cholecystitis.

Intrauterine aortic valvuloplasty in fetuses with critical aortic stenosis: experience and results of 24 procedures.

OBJECTIVE: Valvuloplasty of the fetal aortic valve has the potential to prevent progression of critical aortic stenosis (AS) to hypoplastic left heart syndrome (HLHS). The aim of the study was to assess 24 aortic valvuloplasties regarding indications, success rate, procedure-related risks and outcome. METHODS: Between January 2001 and December 2009 we performed 24 aortic valvuloplasties in 23 fetuses with critical AS at a median gestational age of 26 + 4 (range, 21 + 3 to 32 + 5) weeks by a transabdominal ultrasound-guided approach. Four fetuses had hydrops as a late sign of heart failure. RESULTS: In 16/24 procedures (66.7%) corresponding to 16/23 fetuses (69.6%) the procedures were technically successful, with one intrauterine death in this group. After an initial learning curve, success rate improved to 78.6% (11 of the last 14 interventions were successful). In 10 out of the 15 (66.7%) successfully-treated and liveborn fetuses a biventricular circulation could be achieved postnatally. All four fetuses with hydrops had successful interventions, hydrops disappearing within 5 weeks. In 8/24 interventions (33.3%) the aortic valve could not be treated successfully, with intrauterine fetal death in two of these cases. In one fetus a repeat procedure was successful. All surviving fetuses with unsuccessful (n = 5) or no (n = 5) procedure performed developed HLHS until delivery. CONCLUSIONS: Fetal aortic valvuloplasty could be performed successfully in selected fetuses with critical AS and evolving HLHS, with a biventricular outcome in two thirds of the patients. Safety and success rate were dependent on patient selection and the level of experience of the whole interventional team. In fetuses with AS and hydrops, aortic valvuloplasty could reverse end-stage heart failure and hydrops and ensure fetal survival.

Spasm of the gastroepiploic artery used for coronary artery bypass grafting.

The GEA, though similar to the internal mammary artery in size and flow characteristics, is different histologically as well as physiologically. When used as a free graft, the GEA is very vasospastic, which may influence its long-term patency. When it is necessary to use the GEA as a conduit, it may be preferable to use it in situ rather than as a pedicle graft.

Influence of milrinone and norepinephrine on blood flow in canine internal mammary artery grafts.

BACKGROUND: Vasoactive agents are frequently needed in patients undergoing myocardial revascularization. The purpose of this study was to examine blood flow in the internal mammary artery (IMA) during infusion of drugs that are commonly used after myocardial revascularization. METHODS: A canine right heart bypass preparation allowed precise control of cardiac output and blood pressure, which were maintained constant during drug infusion to isolate the effect of the drug on the IMA conduit. The IMA was anastomosed to a ligated left anterior descending coronary artery. Electromagnetic flow probes measured IMA graft flow. RESULTS: Norepinephrine (0.1 microgram.kg-1.min-1) alone and when combined with phentolamine (8:5 ratio) did not alter IMA flow. Milrinone increased IMA flow 33% +/- 9%, from 37 +/- 7 to 49 +/- 10 mL/min. All hemodynamic variables were unchanged. CONCLUSIONS: The results suggest that: (1) norepinephrine did not have a deleterious effect on IMA flow and (2) milrinone may be a useful drug in patients undergoing myocardial revascularization by increasing IMA blood flow.

Influence of ascending versus descending balloon counterpulsation on bypass graft blood flow.

BACKGROUND: Mechanical circulatory assistance is frequently needed in postcardiotomy patients to support the failing heart. A balloon catheter called SupraCor (ABIOMED Cardiovascular, Inc, Danvers, MA) has been developed and is similar to the existing intraaortic balloon pump with the exception of placement in the ascending versus the descending thoracic aorta. This investigation compared the effects of SupraCor versus standard intraaortic balloon pump on internal mammary artery and venous conduit bypass graft blood flow. METHODS: Porcine total heart bypass was used to anastomose a jugular vein from the ascending aorta to a subsequently ligated left anterior descending coronary artery. The left internal mammary artery was then anastomosed to the jugular vein so each conduit perfused the same coronary vascular bed. An additional right heart bypass preparation allowed precise control of cardiac output and blood pressure, which were maintained constant throughout mechanical circulatory assistance. Electromagnetic flow probes measured flow through each bypass graft and the other conduit was atraumatically occluded. RESULTS: The SupraCor caused a significant increase in both internal mammary artery (+70% from 35 +/- mL/min to 56 +/- 9 mL/min; p = 0.04) and venous bypass graft blood flow (+49% from 66 +/- 12 mL/min to 95 +/- 15 mL/min; p = 0.02) when compared with controls. The intraaortic balloon pump failed to alter internal mammary artery or venous bypass graft flow. CONCLUSIONS: The results demonstrate that counterpulsation with an ascending aortic balloon significantly increases coronary bypass graft flow in both internal mammary artery and venous conduits. In contrast, counterpulsation with a descending aortic balloon did not alter coronary bypass graft flow.

Membranous tracheobronchial injury repaired with gastric serosal patch.

This unusual case involves pharyngolaryngoesophagectomy complicated by injury to the membranous trachea and right bronchus. Repair was possible after partial sternal split and elevation of the tracheostoma through the anterior mediastinum, pulling the stomach to the neck, and using the stomach as a patch to repair the injury to the membranous portion of the airway.

Bone mineral density of the lumbar spine and proximal femur is decreased in children with sickle cell anemia.

Bone mineral density (BMD) was evaluated in the proximal femora (femoral neck, Ward's triangle, and greater trochanter) and lumbar spines of 25 black children and young adults with sickle cell anemia using dual-photon absorptiometry. Compared with normal subjects from the general population, the patients with sickle cell anemia exhibited lower bone mineral density values in all scan regions (approximately 6% to 21% lower than expected). These differences in the lumbar spine were significant for both girls and boys. When compared with normal black subjects from the general population, the girls with sickle cell anemia exhibited significantly lower lumbar spine bone mineral density, and the boys with sickle cell anemia exhibited significantly lower bone mineral density in the femoral neck and Ward's triangle. No consistent or significant correlations were found between the bone mineral density data and the patients' hematologic indices.

[Emergencies in pediatric cardiology]. / Kinderkardiologische Notfälle.

Congenital heart defects, not recognized antenatally, may lead to emergency situation immediately after birth. Fast recognition of clinical symptoms and the use of correct diagnostic tools are life-saving under these circumstances. Principles of cardio-pulmonary resuscitation will be presented in this article as well as diagnosis and treatment of hypoxemic spells, pulmonary hypertensive crises and (usually postoperative) pericardial effusion.

Balloon valvuloplasty in the treatment of congenital aortic valve stenosis--a retrospective multicenter survey of more than 1000 patients.

BACKGROUND: The value of balloon valvuloplasty of the aortic valve in childhood is still under debate. OBJECTIVE: To evaluate the results of the procedure in a retrospective multicenter survey of a large cohort over a long time interval. METHODS: Retrospective analysis of 1004 patients with balloon valvuloplasty of the aortic valve performed between 9/1985 and 10/2006 at 20 centers in Germany, Austria and Switzerland. Amongst others, the following parameters were evaluated before and after the procedure as well as at the end of follow-up or before surgery: clinical status, left ventricular function, transaortic pressure gradient, degree of aortic regurgitation, freedom from re-intervention or surgery. PATIENTS: Patients from 1 day to 18 years of age with aortic valve stenosis were divided into four groups: 334 newborns (1-28 days); 249 infants (29-365 days); 211 children (1-10 years), and 210 adolescents (10-18 years). RESULTS: Median follow-up was 32 months (0 days to 17.5 years). After dilatation the pressure gradient decreased from 65 (± 24)mm Hg to 26 (± 16)mm Hg and remained stable during follow-up. The newborns were the most affected patients. Approximately 60% of them had clinical symptoms and impaired left ventricular function before intervention. Complication rate was 15% in newborns, 11% in infants and 6% in older children. Independently of age, 50% of all patients were free from surgery 10 years after intervention. CONCLUSIONS: In this retrospective multicenter study, balloon valvuloplasty of the aortic valve has effectively postponed the need for surgery in infants, children and adolescents up to 18 years of age.