Prostaglandin E1 Infusion Research Articles

In utero fetal left ventricular rupture and pseudoaneurysm formation: a case report

BackgroundCardiac ventricular aneurysms affect 1 in 200,000 live births. To the best of our knowledge, no reported cases of a left ventricular pseudoaneurym and in utero rupture exist to guide optimal management.Case presentationWe present a case of fetal left ventricular rupture with a large pericardial effusion, cardiac tamponade and subsequent pseudoaneurysm formation with concerns for a poor prognosis. Interventional drainage of the pericardial effusion led to resolution of tamponade and significant improvement in fetal condition. A multidisciplinary team was utilised to plan birth to minimise risk of pseudoaneurysmal rupture and a catastrophic bleed at birth.ConclusionFor similar cases we recommend consideration of birth by caesarean section, delayed cord clamping and a prostaglandin E1 infusion, to reduce the systemic pressures on the left ventricle during transition from fetal to neonatal circulations, until definitive surgical repair. In this case, this resulted in a successful outcome.

The Use of Chronic Total Occlusion (CTO) Wires for Perforation of Atretic Pulmonary Valve; Two Centers Experience

Pulmonary valve atresia with intact ventricular septum (PA-IVS) can be treated either surgically or transcatheterly for eligible patients. Perforation of pulmonary valves using chronic total occlusion (CTO) guidewires has been reported as an alternative to radiofrequency (RF) perforation. We sought to report our experience with CTO guidewires for perforation of atretic pulmonary valves and subsequent balloon dilatation (with or without patent ductus arteriosus stenting) in patients with PA-IVS from two centers. A retrospective study was carried out on PA-IVS patients who underwent intervention between March 2014 and September 2019, in which CTO guidewire was employed for pulmonary valve perforation. A total of 26 patients were identified. The median age and weight of the patients were 5.2days (range 1-21days) and 3.1kg (range 2.2-3.8kg), respectively. All patients were situs solitus, except one patient with left atrial isomerism. The right ventricle (RV) morphology was bipartite in 22/26 patients and tripartite in 4/26 patients. Before the procedure, the mean saturation was 76% (range 70-86%) while the patients were under prostaglandin infusion. The pulmonary valve perforation attempt was performed with the Asahi Conquest Pro 9 CTO wire (n = 6) or Asahi Pro 12 CTO wire (n = 18) and/or Asahi Miracle CTO wire (n = 2). The procedure was successful in 20/26 (77%) patients using CTO wires. We analyzed the efficiency of CTO wire based on the subtypes: Conquest Pro 9 in 6/6 (100%) patients, Conquest Pro 12 in 12/18 (67%) patients, and Miracle in 2/2 (100%) patients. Before CTO wire usage in 3 patients, radiofrequency (RF) perforation was unsuccessful. Among these 3 patients, pulmonary valve perforation was successful in 2 patients with CTO wire; hence, in the remaining patient, perforation was also unsuccessful with CTO wire. After CTO wire perforation was unsuccessful in 6 patients, RF perforation was attempted in 3 patients (2 successful attempts and 1 unsuccessful attempts), and one patient as referred to surgery. Desaturation was persistent in 19 cases, which necessitated ductus arteriosus stenting. Early procedural complication was observed in 3/26 (11%) patients. Two of these patients had vascular complications due to the sheath, which was treated with heparin infusion and streptokinase, and the remaining patient had sudden bradycardia and cardiac arrest during the procedure and did not respond to cardiac resuscitation. CTO wires should be keep in mind for atretic pulmonary valve perforation as a first choice or when RF perforation is unsuccessful.

False Negative Critical Congenital Heart Disease Screening Result Arising from a Complex Cardiac Disease with Duct Dependent Systemic Circulation

Background: Critical congenital heart disease (CCHD) encompasses congenital structural heart defects that cause significant morbidity and mortality in the first few weeks of life unless treated and/or require surgery or catheter intervention within the 1st year of life. Since these deteriorate acutely due to their cardiac condition, they may be misdiagnosed as septicemia or perinatal asphyxia, especially in resource-poor settings. The American Academy of Pediatrics recommends universal screening with pulse oximetry after 24 h of life by a simple screening protocol. Although specificity is high, CCHD may be missed. We present a case who screened negative became symptomatic on day 10 of life and was finally diagnosed with a CCHD. Clinical Description: A full-term baby with uneventful postnatal course and negative CCHD screening was discharged on day 2 of life. He returned on day 10 with cardiogenic shock. Echocardiography confirmed interrupted aortic arch with large ventricular septal defect (VSD), moderate-sized atrial septal defect (ASD), and a small, restrictive patent ductus arteriosus (PDA). The initial false-negative result was attributed to the presence of large VSD that leads to equalization of preductal and postductal oxygen saturations. Management: The baby was stabilized with prostaglandin infusion and ventilatory support. He underwent staged repair with end-to-end anastomosis of interrupted segment and PDA ligation in the first sitting. The postoperative course was uneventful, and the patient was discharged home at day 25 of life. He is planned for VSD and ASD repair in follow-up. Conclusion: Complex heart diseases may behave unusually due to complicated inter-related hemodynamics arising from the various lesions. Primary health-care personnel should recognize the limitations of CCHD screening protocol and learn to counsel parents accordingly.

Enteral Nutrition in Term Infants with Congenital Heart Disease: Knowledge Gaps and Future Directions to Improve Clinical Practice.

Optimal nutrition is essential to improve short- and long-term outcomes in newborns with congenital heart disease (CHD). Nevertheless, several issues on nutritional management and concerns about the potential risk of complications related to enteral feeding exist. This narrative review aims to summarize and discuss the available literature on enteral feeding in term infants with CHD. A wide variability in feeding management exists worldwide. Emerging approaches to improve nutritional status and outcomes in infants with CHD include: implementation of a standardized enteral feeding protocol, both preoperative and postoperative, clearly defining time of initiation and advancement of enteral feeds, reasons to withhold, and definitions of feeding intolerance; early minimal enteral feeding; enteral feeding in stable term infants on hemodynamic support; evaluation of enteral feeding in term infants with umbilical arterial catheters and during prostaglandin infusion; assessment and support of oro-motor skills; and promotion and support of breastfeeding and provision of mother’s own milk or donor milk when mother’s own milk is not available. As evidence from term infants is scarce, available observations and recommendations partially rely on studies in preterm infants. Thus, well-designed studies assessing standardized clinically relevant outcomes are needed to provide robust evidence and shared recommendations and practices.

Early detection of significant congenital heart disease: The contribution of fetal cardiac ultrasound and newborn pulse oximetry screening.

Fetal cardiac and newborn pulse oximetry screening has greatly facilitated the detection of cardiac abnormalities, which may be serious with potentially dire neonatal consequences. The prenatal diagnosis of a serious cardiac abnormality allows the attending obstetrician to organise the much safer in-utero transfer of the fetus for delivery at a tertiary centre, particularly if there is evidence of a duct-dependent lesion that may require the infusion of Prostaglandin E1 to maintain duct patency pending surgical intervention. Newborn pulse oximetry alerts the paediatrician that the baby may have a significant cardiac abnormality, which warrants further elucidation prior to discharge, rather than for the baby to represent unwell a few days later. Despite these advances, serious cardiac abnormalities may be missed on screening. Their detection then falls back onto the clinical acumen of the attending paediatrician/family physician to review the history, carefully elicit and evaluate the clinical signs further aided by whatever investigations that may be available at the birthing hospital, frequently less resourced than the tertiary centres. At the outset, a brief synopsis is provided of the clinical findings that may point to a cardiac abnormality. That is followed by a critical review of the accuracy of prenatal and newborn pulse oximetry screening with emphasis on the lesions that may be missed. Suggestions are made as to how to improve the diagnostic accuracy.

Management of “Wall to Wall Heart” in a Transient Neonatal Tricuspid Regurgitation

We present a case of a one-day-old newborn, without prenatal diagnosis, referred to our cardiologic intensive care unit in critical condition presenting sub-cyanosis and peripheral oxygen saturation of 80%. Echocardiography diagnosis was tricuspid valve dysplasia with severe regurgitation, functional pulmonary valve atresia with intact ventricular septum and reversal flow in the large patent ductus arteriosus (PDA). Chest X-ray showed severe cardiomegaly and wall to wall heart. Prostaglandin E1 infusion was started once after birth. After few days, clinical conditions progressively worsened because of right heart failure; a first pharmacological approach to close PDA failed and surgery ligation of PDA was necessary to restore anterograde pulmonary flow and heart size.

Complex Heart Disease with Interrupted Aortic Arch

Background: Congenital heart disease (CHD) is the most common of all birth defects. Interrupted aortic arch (IAA) is rare, attributing to 1% of all CHD and exhibits left sidedness in most cases. It usually presents in the first 2 weeks of life with heart failure and shock. Early recognition, preoperative stabilization with prostaglandin infusion, and timely surgery are necessary to prevent mortality. Single-stage repair has proven to have good outcomes. However, resource constraints in low- and middle-income countries can hinder diagnosis and intervention by single-stage repair due to a paucity of state of the art centers and inventory. Case Description: We present the experience of a low-resource center with two children with rare complex forms of IAA, both of whom underwent successful single-stage cardiac surgeries; a 6-week-old infant was admitted with difficulty in feeding and heart failure signs. Moreover, a 2-year-old boy was referred with failure to thrive, chronic respiratory problems, and feeding problems since early infancy. Both children were diagnosed with IAA with associated defects including patent ductus arteriosus by transthoracic echocardiography. Management: After stabilization with anticongestive medication, single-stage repair was attempted in both. The postoperative course was uneventful, and the postsurgical echocardiogram showed no residual defect. Both cases were discharged. They are under follow-up, are symptom free, have gained weight, and have not shown any need for reintervention yet. Conclusion: If preparative diagnosis is made correctly and surgery planned meticulously, the immediate and short-term outcome of single-stage repair can be favorable.

Critical Congenital Heart Disease in Newborn: Early Detection, Diagnosis, and Management

Critical congenital heart disease (CHD) is a type of CHD that requires early intervention in the first year of life to survive. Morbidity and mortality increases significantly if newborns with critical CHD experience delay in the initial diagnosis and management. The infants may develop cyanosis, systemic hypoperfusion, or respiratory distress as the main manifestations of critical CHD. Pulse oximetry screening for early detection of critical CHD must be performed in newborns after 24 hours of age or before discharge from hospital. Generally, infants with critical CHD require patency of the ductus arteriosus with infusion of prostaglandin to maintain pulmonary or systemic blood flow. After initial management, the infants must be immediately referred to tertiary care center for definitive intervention.
 Keywords: congenital heart disease, duct-dependent circulation, ductus arteriosus, prostaglandin

Digital ischaemia of the upper limbs in middle age: consider arterial thoracic outlet syndrome until proven otherwise!

ObjectivesPatients presenting with digital upper limb ischaemia are occasionally referred to rheumatology services to rule out vasculitis. We aimed to present two cases of delayed diagnosis of arterial thoracic outlet syndrome (aTOS) in middle-aged patients presenting with digital ischaemia in order to raise awareness of this important pathology that requires timely surgical intervention.MethodsTwo cases of progressive ischaemia of the right upper extremity caused by primarily undiagnosed compression of the subclavian artery by an accessory cervical rib are presented. The case notes, radiological images, intra-operative and postoperative findings for both patients were reviewed. Patients were followed up after ≥6 months to assess prognosis.ResultsBoth patients had a working diagnosis of Buerger’s disease and had been treated with prostaglandin infusions before establishment of the diagnosis of arterial thoracic outlet syndrome. Both patients were heavy smokers, and one patient had bilateral symptoms and a history of axial SpA and positive HLA-B27. Late presentation in one patient led to the loss of three fingers and the need for plastic reconstructive surgery after cervical rib resection and revascularization. In the other patient, surgical thrombectomy of the upper limb arteries along with resection of a cervical rib and repair of the subclavian artery with an interposition graft were necessitated to heal digital ulcers successfully.ConclusionA high index of suspicion of aTOS should be maintained in middle-aged patients presenting with digital or upper limb ischaemia even in the presence bilateral symptoms or relevant risk factors of other diagnoses, such as smoking or a positive rheumatological history.

Umbilical vein infusion of prostaglandin I2 increases ductus venosus shunting of oxygen-rich blood but does not increase cerebral oxygen delivery in the fetal sheep.

The ductus venosus (DV) is a dynamic fetal shunt that allows substrate-rich blood from the umbilical vein to bypass the hepatic circulation. In vitro studies suggest a direct role of prostaglandin I2 (PGI2 ) in the regulation of DV tone; however, the extent of this regulation has not been determined in utero. 4D flow and T2 oximetry magnetic resonance imaging can be combined to determine blood flow and oxygen delivery within the fetal circulation. PGI2 increases DV shunting of substrate-rich blood but this does not increase cerebral oxygen delivery. During fetal development, the maintenance of adequate oxygen and nutrient supply to vital organs is regulated through specialized fetal shunts. One of these shunts, the ductus venosus (DV), allows oxygen-rich blood to preferentially stream from the placenta toward the heart and brain. Herein, we combine magnetic resonance imaging (MRI) techniques that measure blood flow (4D flow) and oxygen saturation (T2 oximetry) in the fetal circuit to determine whether umbilical vein infusion of prostaglandin I2 (PGI2 , regulator of DV tone ex utero) directly dilates the DV and thus increases the preferential streaming of oxygen-rich blood toward the brain. At 114-115 days gestational age (dGA; term = 150 days), fetal sheep (n = 6) underwent surgery to implant vascular catheters in the fetal femoral artery, femoral vein, amniotic cavity and umbilical vein. Fetal MRI scans were performed at 119-124 dGA. 4D flow and T2 oximetry were performed to measure blood flow and oxygen saturation across the fetal circulation in both a basal state and whilst the fetus was receiving a continuous infusion of PGI2 . The proportion of oxygenated blood that passed through the DV from the umbilical vein was increased by PGI2 . Cerebral oxygen delivery was unchanged in the PGI2 state. This may be a result of decreased flow from the right to left side of the heart as blood flow through the foramen ovale was decreased by PGI2 . We have shown that although PGI2 acts on the DV to increase the proportion of oxygen-rich blood that bypasses the liver, this does not increase cerebral oxygen delivery in the fetal sheep.

Improving diagnostic accuracy in neonates with left heart obstruction in a transport setting

Differentiating left heart obstruction (LHO) from other severe illness in the neonatal period is challenging, and important for guiding clinical management. The aim of this study was to identify factors distinguishing LHO from non-LHO in neonates. A retrospective, cohort study of neonates referred to the Newborn and Paediatric Emergency Transport Service, New South Wales, with suspected LHO during the epoch 1996-2016. A total of 273 neonates were included; 240 with confirmed LHO. Administration of prostaglandin E1 to infants with a structurally normal heart was not associated with impaired acid-base or oxygenation status. Pre-transport diagnostic accuracy of LHO was 74.4%; sensitivity 84.5%, positive predictive value 86.0%. On multivariable logistic regression, hepatomegaly (odds ratio 2.54; 95% confidence interval 1.05-6.16) was associated with confirmed LHO. A low threshold for prostaglandin E1 infusion should be maintained in infants with suspected LHO. Hepatomegaly is associated with a diagnosis of LHO and may be more useful than other parameters in predicting the condition.

Balloon valvuloplasty for critical pulmonary valve stenosis in a newborn

Abstract Critical pulmonary valvar stenosis causes cyanosis and can lead to potentially lethal in newborn infants. Following initiation of Prostaglandin infusion, percutaneous balloon pulmonary valvuloplasty (BPV) is considered to be the treatment of choice. The percutaneous femoral venous approach is the most preferred route for BPV in most cardiac centers. We present the case of a 5-day-old female infant with critical pulmonary valvar stenosis who successfully underwent BPV through the femoral venous access. The narrowed pulmonary valve was significantly improved after intervention. This was the first case at our center that was undertaken BPV at the earliest postnatal day.

Intravenous infusion of prostaglandin E1 in ischemic ulcer and rest pain for extremities due to peripheral vascular disease

Prostaglandins are potent vasoactive agents with wide variety of other actions - vasodilatation, fibrinolysis and inhibition of platelet aggregation. PGE1 was the agent used since 1973 for cardiovascular diseases, mainly in patients with advanced PVD. PGE1 intra venous infusion has shown to be beneficial in limb threatening ischemia, especially when reconstructive procedures are not feasible. The original research of benefitted 35 cases in last 5 years is presented here. Background: As a General surgeon we use only analgesics and anti-platelet agent aspirin for severe ischaemic limbs with rest pain. We could not improve patient’s quality of life with these medications. Enough of medicine literature says prostaglandins E1 improves microcirculation, stop tissue damage due to ischemia and relief from rest pain. Methods: In this study we included both gender patients above age of 34 years who had ischemic changes associated with peripheral vascular disease and rest pain. We infused intravenous prostaglandins E1 and studied them for improvement in soft tissue ischemic changes and in rest pain. Results: clinical success was evaluated according subjective grading and relief of pain. Patients in these studies had a pain score from 6 onwards as per Wong-Baker FACES pain rating scale improved to 2-4. We also studied them for Fontain and Rutherford staging and scoring system, which improved from III b to II-I and from 4 to 2-1 respectively. Conclusions: Intravenous infusion of PGE1 alprostadil on 5 days a cycle basis, in selected patients with severe rest pain and ischemia seems to be very effective, without any serious complications.

Genotype\u2013phenotype correlation in two Polish neonates with alveolar capillary dysplasia

BackgroundAlveolar capillary dysplasia (ACD) is a rare cause of severe pulmonary hypertension and respiratory failure in neonates. The onset of ACD is usually preceded by a short asymptomatic period. The condition is refractory to all available therapies as it irreversibly affects development of the capillary bed in the lungs. The diagnosis of ACD is based on histopathological evaluation of lung biopsy or autopsy tissue or genetic testing of FOXF1 on chromosome 16q24.1. Here, we describe the first two Polish patients with ACD confirmed by histopathological and genetic examination.Case presentationThe patients were term neonates with high Apgar scores in the first minutes of life. They both were diagnosed prenatally with heart defects. Additionally, the first patient presented with omphalocele. The neonate slightly deteriorated around 12th hour of life, but underwent surgical repair of omphalocele followed by mechanical ventilation. Due to further deterioration, therapy included inhaled nitric oxide (iNO), inotropes and surfactant administration. The second patient was treated with prostaglandin E1 since birth due to suspicion of aortic coarctation (CoA). After ruling out CoA in the 3rd day of life, infusion of prostaglandin E1 was discountinued and immediately patient’s condition worsened. Subsequent treatment included re-administration of prostaglandin E1, iNO and mechanical ventilation. Both patients presented with transient improvement after application of iNO, but died despite maximized therapy. They were histopathologically diagnosed post-mortem with ACD. Array comparative genomic hybridization in patient one and patient two revealed copy-number variant (CNV) deletions, respectively, ~ 1.45 Mb in size involving FOXF1 and an ~ 0.7 Mb in size involving FOXF1 enhancer and leaving FOXF1 intact.ConclusionsBoth patients presented with a distinct course of ACD, extra-pulmonary manifestations and response to medications. Surgery and ceasing of prostaglandin E1 infusion should be considered as potential causes of this variability. We further highlight the necessity of thorough genetic testing and histopathological examination and propose immunostaining for CD31 and CD34 to facilitate the diagnostic process for better management of infants with ACD.

Caffeine prevents prostaglandin E1-induced disturbances in respiratory control in neonatal rats: implications for infants with critical congenital heart disease.

Continuous infusion of prostaglandin E1 (PGE1) is used to maintain ductus arteriosus patency in infants with critical congenital heart disease, but it can also cause central apnea suggesting an effect on respiratory neural control. In this study, we investigated whether 1) PGE1 inhibits the various phases of the acute hypoxic ventilatory response (HVR; an index of respiratory control dysfunction) and increases apnea incidence in neonatal rats; and 2) whether these changes would be reversible with caffeine pretreatment. Whole body plethysmography was used to assess the HVR and apnea incidence in neonatal rats 2 h following a single bolus intraperitoneal injection of PGE1 with and without prior caffeine treatment. Untreated rats exhibited a biphasic HVR characterized by an initial increase in minute ventilation followed by a ventilatory decline of the late phase (~5th minute) of the HVR. PGE1 had a dose-dependent effect on the HVR. Contrary to our hypothesis, the lowest dose (1 µg/kg) of PGE1 prevented the ventilatory decline of the late phase of the HVR. However, PGE1 tended to increase postsigh apnea incidence and the coefficient of variability (CV) of breathing frequency, suggesting increased respiratory instability. PGE1 also decreased brainstem microglia mRNA and increased neuronal nitric oxide synthase (nNOS) and platelet-derived growth factor-β (PDGF-β) gene expression. Caffeine pretreatment prevented these effects of PGE1, and the adenosine A2A receptor inhibitor MSX-3 had similar preventative effects. Prostaglandin appears to have deleterious effects on brainstem respiratory control regions, possibly involving a microglial-dependent mechanism. The compensatory effects of caffeine or MSX-3 treatment raises the question of whether prostaglandin may also operate on an adenosine-dependent pathway.

Cannulation of the external iliac artery in a newborn with interrupted aortic arch and premature closure of the ductus

Abstract After an uneventful vaginal delivery at term, the baby deteriorated within a few minutes and required intubation. Echocardiography showed an interrupted aortic arch with no significant ductus blood flow. Prostaglandin infusion improved oxygenation temporarily. Rising lactate levels indicated inadequate tissue oxygenation. In spite of inotropic support, several episodes of bradycardia requiring CPR occurred. At ten hours of life, the baby was cannulated for veno-arterial ECMO with drainage from the right internal jugular vein. In order to ensure perfusion in the lower body, blood was infused via an arterial cannula inserted in the right external iliac artery. The patient was transferred on ECMO to a pediatric surgical heart center. After stabilization, the aortic malformation was repaired via a left thoracotomy during hypothermia with the same cannulation, the baby was decannulated and the iliac artery repaired. At four weeks of age, the baby was discharged home on room air and full enteral feeding. It is concluded that arterial cannulation of the external iliac artery for ECMO in a newborn is an alternative, if exclusive perfusion of the lower body is desired or if the right carotid artery is unavailable.

Foramen ovale and ductus arteriosus hemodynamics in Dextro Transposition of Great Arteries (D-TGA) with intact ventricular septum, case report, and literature review

D-TGA is characterized by discordant Ventriculo-arterial connection. For the patient to survive there should be a mixing of the oxygenated and deoxygenated blood at the atrial, ventricular and/or the great vessel level. The hemodynamics of the PFO and ductus arteriosus in D-TGA with intact ventricular septum (VS) is complex and depends upon the relative difference between the pulmonary and systemic vascular resistance as well as the ventricular compliance. D-TGA with intact VS might be associated with persistent pulmonary hypertension as well as with low pulmonary vascular resistance leading to changes in the hemodynamics and direction of flow through the PDA and/or the PFO. The indications of balloon atrial septestomy as well as the initiation and/or the continuation of Prostaglandin infusion are conflicting issues in such cases. We are presenting a case of D-TGA with intact VS who developed steeling and signs of low systemic blood flow because of a large PDA. We reviewed the hemodynamics of PFO and DA in such cases as well as the indications of BAS and PG infusion.

Effect of Long-term Administration of Prostaglandin E1 on Morphologic Changes in Ductus Arteriosus

To improve survival of patients with hypoplastic left heart syndrome, combination therapy with bilateral pulmonary artery banding and prostaglandin E1 (PGE1)-mediated ductal patency was developed as an alternative for high-risk neonates in Japan. However, the effect of long-term PGE1 administration on ductus arteriosus remains unclear. Synchrotron radiation-based X-ray phase-contrast tomography (XPCT) enables clear visualization of soft tissues at an approximate spatial resolution of 12.5 μm. We aimed to investigate morphologic changes in ductus arteriosus after long-term PGE1 infusion using XPCT. Seventeen ductus arteriosus tissue samples from patients with hypoplastic left heart syndrome were obtained during the Norwood procedure. The median duration of lipo-prostaglandin E1 (lipo-PGE1) administration was 48 days (range, 3 to 123). Structural analysis of ductus arteriosus was performed and compared with conventional histologic analysis. The XPCT was successfully applied to quantitative measurements of ductal media. Significant correlation was found between the duration of lipo-PGE1 infusion and mass density of ductal media (R= 0.723, P= .001). The duration of lipo-PGE1 administration was positively correlated with elastic fiber staining (R= 0.799, P < .001) and negatively correlated with smooth muscle formation (R=-0.83, P < .001). No significant increase in intimal cushion formation was found after long-term lipo-PGE1 administration. Expression of ductus arteriosus dominant PGE2-receptor EP4 almost disappeared in specimens when lipo-PGE1 was administered over 3 days. Disorganized elastogenesis and little intimal cushion formation after long-term lipo-PGE1 administration suggest that ductus arteriosus remodeled to the elastic artery phenotype. Because EP4 was downregulated and ductus arteriosus exhibited elastic characteristics, the dosage of lipo-PGE1 might be decreased after a definite administration period.

Staged surgical repair for infants with interrupted aortic arch

BackgroundInterrupted aortic arch (IAA) is a rare congenital cardiac anomaly, which necessitates surgical treatment. There are several surgical strategies for corrective repair of IAA, such as one-stage repair, rapid two-stage repair and two-stage repair. Here, we reported our surgical result of staged-repair policy for the patients with IAA. MethodFrom November 2003 to July 2015, there were 14 patients (8 boys, 6 girls) with IAA treated by us. Except one teenager patient, we routinely used intravenous infusion of prostaglandin E1 for all the infant patients (n = 13) to keep adequate end organ perfusion before the first surgical intervention. Surgical repair was performed after the perfusion of end organs recovered. ResultTwo patients (1 teenager and 1 infant with one-stage surgery) were excluded from this study. At the time of the first surgery, we did the first-stage surgery with anastomosis in between aortic arch and descending aorta, division of patent ductus arteriosus and banding of pulmonary trunk through left thoracotomy. The overall surgical survival rate of the first surgery was 100% (12/12). At the time of the second surgery, corrective repair was done under cardiopulmonary bypass through median sternotomy. The surgical survival rate of the corrective surgery was also 100%. There is no late death during follow-up for 9 years (range 4.2–15.0 years). ConclusionOut of several surgical strategies for the infants with IAA, staged repair still could be a treatment option to achieve satisfied surgical result.

Ciężkie zwężenie zastawki aortalnej u noworodka

Aortic stenosis is a congenital heart disease which may present with different symptomatology and may lead to heart failure in newborn. A case of a 9-days old newborn with a severe aortic stenosis is presented. Due to impaired systolic function of the left ventricle and rapidly increasing symptoms of heart failure (tachypnea, dyspnoea, tachycardia) the neonate required pharmacological treatment (prostaglandin E1 infusion and phosphodiesterase inhibitor c-AMP) and balloon valvuloplasty as a matter of urgency. In chest X-ray the heart silhouette was enlarged, the electrocardiogram showed signs of overload of both ventricles. In echocardiography thickened aortic valves with a pin-hole opening, normal left ventricle size with signs of its hypertrophy and reduced contractility (ejection fraction 45%) and a moderate mitral regurgitation were found. Angiography and balloon valvuloplasty of the aortic valve was performed through puncture of the right femoral artery. After expansion of the balloon at the stenotic valve a very good result of the treatment was achieved – low invasive gradient (22 mm Hg) between the left ventricle and the aorta and a trivial aortic valve regurgitation. The good outcome of the procedure was confirmed and monitored in the next days. The general state of the child improved significantly, systolic function of the left ventricle returned to normal values. Because of a complication after the procedure – a thrombosis in the external iliac artery, a 3-month anti-clotting therapy was performed. No improvement in the patency of the vessel was observed; however, abundant collateral vessels provided the extremity with proper circulation.