Patency Of The Ductus Arteriosus Research Articles

Many Term infants with Persistent Patency of the Ductus Arteriosus could be Trisomy 21 Mosaics

Many Term infants with Persistent Patency of the Ductus Arteriosus could be Trisomy 21 Mosaics

Access to emergency surgical treatment for congenital heart diseases in the state of São Paulo

ObjectiveTo analyze access to surgical care for congenital heart diseases in public specialized centers in the state of São Paulo before and during the COVID-19 pandemic and availability of surgical care in specialized hospitals, to identify bottlenecks in the care path for neonatal cardiac surgery. MethodsThis study included 1,437 children, under one year old, with congenital heart disease and formal referral to heart surgery between February 1, 2019, and February 28, 2021. Quantitative data analysis was performed using t-tests or the Mann-Whitney test. ResultsApproximately 30 % of children with urgent congenital heart disease could not access recommended care, mainly those needing complex surgeries (categories RACHS 4 to 6). The main diagnoses of neonates accessing care were patency of the ductus arteriosus (10.5 %) and coarctation of the aorta (10.1 %). Referral time for children in RACHS 1 to 3 was 4 days (median), while for those in categories 4 to 6, it was 7 days (p < 0.001). Longer referral time (20 days) was associated with court orders. During the pandemic, referral time decreased to 3 days, compared to 5 days (median) in the pre-pandemic period (p < 0.001). ConclusionThe emergency surgical treatment supply for congenital heart diseases is insufficient compared to the current demand. Future research should evaluate if access to care in publicly funded hospitals could be improved by better access to prenatal care for pregnant women, increased diagnostic and therapeutic capacity in pediatric cardiology, and financial incentives for complex cardiac surgeries.

A novel role for PGE2-EP4 in the developmental programming of the mouse ductus arteriosus: consequences for vessel maturation and function.

The ductus arteriosus (DA) is a vascular shunt that allows oxygenated blood to bypass the developing lungs in utero. Fetal DA patency requires vasodilatory signaling via the prostaglandin E2 (PGE2) receptor EP4. However, in humans and mice, disrupted PGE2-EP4 signaling in utero causes unexpected patency of the DA (PDA) after birth, suggesting another role for EP4 during development. We used EP4-knockout (KO) mice and acute versus chronic pharmacological approaches to investigate EP4 signaling in DA development and function. Expression analyses identified EP4 as the primary EP receptor in the DA from midgestation to term; inhibitor studies verified EP4 as the primary dilator during this period. Chronic antagonism recapitulated the EP4 KO phenotype and revealed a narrow developmental window when EP4 stimulation is required for postnatal DA closure. Myography studies indicate that despite reduced contractile properties, the EP4 KO DA maintains an intact oxygen response. In newborns, hyperoxia constricted the EP4 KO DA but survival was not improved, and permanent remodeling was disrupted. Vasomotion and increased nitric oxide (NO) sensitivity in the EP4 KO DA suggest incomplete DA development. Analysis of DA maturity markers confirmed a partially immature EP4 KO DA phenotype. Together, our data suggest that EP4 signaling in late gestation plays a key developmental role in establishing a functional term DA. When disrupted in EP4 KO mice, the postnatal DA exhibits signaling and contractile properties characteristic of an immature DA, including impairments in the first, muscular phase of DA closure, in addition to known abnormalities in the second permanent remodeling phase.NEW & NOTEWORTHY EP4 is the primary EP receptor in the ductus arteriosus (DA) and is critical during late gestation for its development and eventual closure. The "paradoxical" patent DA (PDA) phenotype of EP4-knockout mice arises from a combination of impaired contractile potential, altered signaling properties, and a failure to remodel associated with an underdeveloped immature vessel. These findings provide new mechanistic insights into women who receive NSAIDs to treat preterm labor, whose infants have unexplained PDA.

Maldevelopment of the Immature Pulmonary Vasculature and Prolonged Patency of the Ductus Arteriosus - Association or Cause?

"Maldevelopment of the Immature Pulmonary Vasculature and Prolonged Patency of the Ductus Arteriosus – Association or Cause?." American Journal of Respiratory and Critical Care Medicine, 0(ja), pp. –

Current state of the art in hypoplastic left heart syndrome.

Hypoplastic left heart syndrome (HLHS) is a complex congenital heart condition in which a neonate is born with an underdeveloped left ventricle and associated structures. Without palliative interventions, HLHS is fatal. Treatment typically includes medical management at the time of birth to maintain patency of the ductus arteriosus, followed by three palliative procedures: most commonly the Norwood procedure, bidirectional cavopulmonary shunt, and Fontan procedures. With recent advances in surgical management of HLHS patients, high survival rates are now obtained at tertiary treatment centers, though adverse neurodevelopmental outcomes remain a clinical challenge. While surgical management remains the standard of care for HLHS patients, innovative treatment strategies continue to be developing. Important for the development of new strategies for HLHS patients is an understanding of the genetic basis of this condition. Another investigational strategy being developed for HLHS patients is the injection of stem cells within the myocardium of the right ventricle. Recent innovations in tissue engineering and regenerative medicine promise to provide important tools to both understand the underlying basis of HLHS as well as provide new therapeutic strategies. In this review article, we provide an overview of HLHS, starting with a historical description and progressing through a discussion of the genetics, surgical management, post-surgical outcomes, stem cell therapy, hemodynamics and tissue engineering approaches.

The ductus arteriosus in neonates with critical congenital heart disease.

The ductus arteriosus (DA) has a paradoxical biological role in neonates with congenital heart disease (CHD) and can present with significant management challenges. Critical congenital cardiac lesions rely on the patency of the DA to provide either systemic or pulmonary blood flow. A patent DA (PDA) that remains open can also have adverse consequences depending on the degree of systemic to-pulmonary shunting and volume of ductal steal. As such, the presence of a PDA may pose a challenge in the medical management and timing of surgical repair. In this perspective article, we provide an understanding of the role of the DA in the circulatory system in neonates with CHD and discuss traditional and emerging approaches to support the pulmonary and systemic circulations with manipulation of the DA.

Critical coarctation of the aorta in newborn - A case presentation

Background: Heart defects are the most popular congenital disease noticed among infants. Aortic Coarctation (CoA) forms 5 - 10 % of congenital heart diseases. The heart defect consist on aortic coarctation between inlet of the left subclavian artery and origin of the arterial ligament. In the during neonatal and early infantile period, in case of delayed diagnosis, it may lead to acute heart failure and the death of the child. &#x0D; Case report: A female infant born at 37 weeks of gestation, by caesarean section, in good condition, was admitted to the Neonatal Department. There was a suspicion of CoA in the fetus in the pregnancy history. The Echocardiography showed a narrowing of the carpal tunel, prostin was included in the treatment. In the second day of life, the child was transferred to the Cardiology Department. Four-extremity blood pressures test showed higher values in the upper limbs than in the lower limbs. Echocardiography revealed hypoplasia of the aortic arch in the section below the exit of the second cephalic vessel with coarctation at the isthmus site. Laboratory tests showed a high level of pro-BNP. The control echocardiography showed the anatomy of the defect as before, an unobstructed wide arterial duct with a dominant R-L flow, a flow in the descending aorta of 2.7 m/s, with a coartable flow spectrum. The child was transported to the Department of Cardiac Surgery, Heart Transplantation and Mechanical Circulatory Support for Children in Zabrze for surgical treatment. &#x0D; Conclusions: In neonates and infants with critical aortic stenosis, ensure the patency of the ductus arteriosus with continuous intravenous infusion of prostaglandin E1. Currently, echocardiography is the basic method in the diagnosis of aortic stenosis. &#x0D;

Value of bedside echocardiography in predicting persistent patency of the ductus arteriosus during the early postnatal period in very low birth weight infants.

To study the value of bedside echocardiography in predicting persistent patency of the ductus arteriosus during the early postnatal period in very low birth weight (VLBW) infants. A retrospective analysis was performed for 51 VLBW infants who were admitted from March 2020 to June 2021, with an age of ≤3 days and a length of hospital stay of ≥14 days. According to the diameter of patent ductus arteriosus (PDA) on days 14 and 28 after birth, the infants were divided into three groups: large PDA group (PDA diameter ≥2 mm), small PDA group (PDA diameter <2 mm), and PDA closure group (PDA diameter =0 mm). The echocardiographic parameters measured at 72 hours after birth were compared among the three groups. The receiver operating characteristic (ROC) curve was used to evaluate the value of the echocardiographic parameters in predicting persistent patency of the ductus arteriosus (PDA≥2 mm) at the ages of 14 and 28 days. On day 14 after birth, there were 17 infants in the large PDA group, 11 in the small PDA group, and 23 in the PDA closure group. On day 28 after birth, there were 14 infants in the large PDA group, 9 in the small PDA group, and 26 in the PDA closure group. There were significant differences in gestational age, birth weight, rate of pulmonary surfactant use, and incidence rate of hypotension among the three groups (P<0.05). PDA diameter, end-diastolic velocity of the left pulmonary artery, left ventricular output, and left ventricular output/superior vena cava flow ratio measured at 72 hours after birth were associated with persistent patency of the ductus arteriosus at the ages of 14 and 28 days (P<0.05), and the ratio of the left atrium to aorta diameter was associated with persistent patency of the ductus arteriosus at the age of 28 days (P<0.05). The ROC curve analysis showed that the area under the curve that the PDA diameter measured at 72 hours after birth predicting the persistent patency of the ductus arteriosus at the ages of 14 and 28 days was the largest (0.841 and 0.927 respectively), followed by end-diastolic velocity of the left pulmonary artery, with the area under the curve of 0.793 and 0.833 respectively. The indicators obtained by beside echocardiography at 72 hours after birth, especially PDA diameter and end-diastolic velocity of the left pulmonary artery, can predict persistent patency of the ductus arteriosus at the ages of 14 and 28 days in VLBW infants, which provides a basis for the implementation of early targeted treatment strategy for PDA.

C39. Ductus Arteriosus Stenting: A Promising and Life Saving Procedure for Ductal-Dependent Pulmonary Blood Flow

Abstract Background Duct-dependant congenital heart disease in newborn presents as a life-threatening emergency and the survival depends on patency of the ductus arteriosus. Stent implantation into ductus arteriosus has been proposed as non-surgical management for critical congenital heart disease with duct-dependent. Case Summary We present full-term newborn babies who had critical congenital heart disease with ductal-dependent pulmonary blood blow and successfully managed with ductal stenting. Patient A, a 13-days-old male had Tetralogy of Fallot-Pulmonary Atresia and restrictive Ventricular Septal Defect (VSD). Patient B, a 3-days-old male had Pulmonary Atresia-Intact Ventricular Septum (PA-IVS) with Patent Foramen Ovale (PFO). Patient C, a 5-days-old female had Tricuspid Atresia with restrictive VSD and multiple congenital anomaly. Patient D, a 2-days-old female had PA-IVS with PFO stretch. Prostaglandin-E1 infusion was started at first and stop 6 hours prior to the procedure. Ductus arteriosus stenting using drug-eluting stent and femoral artery approach was successful in all 4 patients. All patient had improvement of oxygen saturation and good post-procedure outcome. Discussion Ductal stenting in patient with duct-dependent pulmonary circulation appears to be an alternative to surgery. It provides a bridging palliation in cyanotic congenital heart disease until the time of definitive surgery. Stenting of ductus arteriosus is feasible and safe with good result, thus early recognition and timely management are important to save the life.

C39. Ductus Arteriosus Stenting: A Promising and Life Saving Procedure for Ductal-Dependent Pulmonary Blood Flow

Abstract Background Duct-dependant congenital heart disease in newborn presents as a life-threatening emergency and the survival depends on patency of the ductus arteriosus. Stent implantation into ductus arteriosus has been proposed as non-surgical management for critical congenital heart disease with duct-dependent. Case Summary We present full-term newborn babies who had critical congenital heart disease with ductal-dependent pulmonary blood blow and successfully managed with ductal stenting. Patient A, a 13-days-old male had Tetralogy of Fallot-Pulmonary Atresia and restrictive Ventricular Septal Defect (VSD). Patient B, a 3-days-old male had Pulmonary Atresia-Intact Ventricular Septum (PA-IVS) with Patent Foramen Ovale (PFO). Patient C, a 5-days-old female had Tricuspid Atresia with restrictive VSD and multiple congenital anomaly. Patient D, a 2-days-old female had PA-IVS with PFO stretch. Prostaglandin-E1 infusion was started at first and stop 6 hours prior to the procedure. Ductus arteriosus stenting using drug-eluting stent and femoral artery approach was successful in all 4 patients. All patient had improvement of oxygen saturation and good post-procedure outcome. Discussion Ductal stenting in patient with duct-dependent pulmonary circulation appears to be an alternative to surgery. It provides a bridging palliation in cyanotic congenital heart disease until the time of definitive surgery. Stenting of ductus arteriosus is feasible and safe with good result, thus early recognition and timely management are important to save the life.

10 High spontaneous ductal closure even at the extreme of prematurity

Abstract Primary Subject area Neonatal-Perinatal Medicine Background Extremely preterm newborns are at risk of prolonged patency of the ductus arteriosus (PDA). Current literature has failed to indicate improvement in outcomes after exposure to strategies promoting ductal closure. As such, our center abandoned these practices in 2013. Objectives Describe the spontaneous PDA closure in premature infants, including those infants born at the extreme of gestational age (&amp;lt; 26 weeks). Design/Methods Retrospective study of newborns &amp;lt; 29 weeks, admitted within 24 hours after birth between 2015 and 2019 and without genetic or congenital anomalies. Newborns who were last known to be alive, with an available echocardiography, and who were not exposed to any intervention to accelerate PDA closure were included. Images were reviewed by experts blinded to the outcomes. Results 296 infants were analyzed. 37 (12%) did not survive their hospitalization, and 16 were exposed to interventions to accelerate ductal closure at some point during their lifetime (4 ligations, 4 catheter-closure, 5 ibuprofen and 3 acetaminophen). Out of the 243 remaining newborns, 214 had at least one echocardiography to ascertain ductal patency or closure (100% of those &amp;lt;26 weeks). The average gestational age was 26.3±1.5 weeks, with 84 (39%) being &amp;lt;26 weeks. PDA closed spontaneously in 194 (91%), with 60 having closure ascertainment after discharge (average age at closure ascertainment of 36.4 [IQR: 34.4 – 40.1] weeks). Of the 84 &amp;lt;26 weeks, 76 (90%) had confirmation of ductal closure. The 20 infants with an open PDA at the last evaluation were followed in an outpatient setting and considered small/restrictive. In our cohort, 92/243 (38%) were exposed to post-natal steroids. In the &amp;lt;26 weeks group, 74% were exposed to steroids, at a cumulative dose of 1.64 [0.89 – 2.44] mg/kg. BPD was found in 57% of the overall cohort and in 79% of &amp;lt;26 weeks. Conclusion The majority of newborns &amp;lt; 29 weeks, and even those at the extreme of gestational age (&amp;lt; 26 weeks) spontaneously closed their PDA before term-corrected age. While BPD rate was similar to previous cohorts, post-natal steroids use was high.

9 The Burden of Bronchopulmonary Dysplasia and Pulmonary Vascular Disease in Premature Infants

Abstract Primary Subject area Neonatal-Perinatal Medicine Background Extremely premature infants are at a high risk of bronchopulmonary dysplasia (BPD) and BPD-associated pulmonary hypertension (PH). Prolonged patency of the ductus arteriosus (PDA) may worsen PH; however, due to the lack of evidence supporting improvement in outcomes after strategies to promote ductal closure, our center has adopted a strict non-intervention policy since 2013. Objectives Assess PH prevalence and severity, as well as the impact of BPD on echocardiographic parameters of cardiac performance. Design/Methods Retrospective cohort of infants &amp;lt;29 weeks gestational age at birth, admitted between 2015 and 2019, and without genetic/congenital anomalies. Measurements from the echocardiography acquired closest to 36 weeks were done by masked experts. Severe BPD was defined as positive pressure support at 36 weeks. PH was defined as an estimated systolic pulmonary pressure (SPAP) ≥40 mmHg or an abnormal septal curvature by eccentricity index (EI) (&amp;gt;1.3). Results Out of 387 infants, 222 were included, of which 27 (12%) were categorized as severe BPD and 78 (35%) had PH. Severe BPD was associated with lower birth weight (704±214 vs 842±229g, p&amp;lt;0.01), longer hospitalization (median 138 [IQR 108-167] vs 103 [IQR 86-125] days, p&amp;lt;0.01) and longer mechanical ventilation duration (median 82 [IQR 33-107] vs 17 [IQR 2-32] days, p&amp;lt;0.01), with no difference in gestational age at birth. Severe BPD was associated with PH (70% vs 43%, p&amp;lt;0.01). The combined outcome of death (after the 36 weeks echocardiography) or severe BPD was associated with PH (68% vs 30%, p&amp;lt;0.01), smaller left ventricle length in diastole (2.8±0.5 vs 3.0±0.5 cm, p=0.03), decrease in the tricuspid annular plane systolic excursion (0.7±0.2 vs 0.9±0.2 cm, p&amp;lt;0.01), abnormal EI (1.31±0.25 vs 1.17±0.18, p&amp;lt;0.01) and smaller right ventricle fraction area change (41.3±5.8 vs 47.8±7.6%, p&amp;lt;0.01), without a significant increase on SPAP (35±21 vs 35±14, p=0.15). Other echocardiographic markers were similar. Conclusion In the context of a PDA non-intervention policy, a third of our population was affected by PH at 36 weeks. Furthermore, those with severe BPD or death had signs of RV dysfunction (despite similar SPAP estimate), indicating that the effect of BPD on pulmonary vascular remodelling and cardiac function may be underestimated.

Mouse models of patent ductus arteriosus (PDA) and their relevance for human PDA.

The ductus arteriosus (DA) is a unique fetal vascular shunt, which allows blood to bypass the developing lungs in utero. After birth, changes in complex signaling pathways lead to constriction and permanent closure of the DA. The persistent patency of the DA (PDA) is a common disorder in preterm infants, yet the underlying causes of PDA are not fully defined. Although limits on the availability of human DA tissues prevent comprehensive studies on the mechanisms of DA function, mouse models have been developed that reveal critical pathways in DA regulation. Over 20 different transgenic models of PDA in mice have been described, with implications for human DA biology. Similarly, we enumerate 224 human single‐gene syndromes that are associated with PDA, including a small subset that consistently feature PDA as a prominent phenotype. Comparison and functional analyses of these genes provide insight into DA development and identify key regulatory pathways that may serve as potential therapeutic targets for the management of PDA.

Anesthesia Management in VP Shunt Surgery in Neonates with Hypoplastic Left Heart Syndrome (HLHS) and Dandy Walker Syndrome

&lt;p&gt;&lt;strong&gt;Introduction:&lt;/strong&gt; Hypoplastic left heart syndrome (HLHS) is defined as the incomplete development of the left heart structures, including the mitral valve, left ventricle, aortic valve, and aortic arch. The clinical presentation of HLHS depends on the patency of the ductus arteriosus and the degree of restriction of the atrial septum. Common clinical manifestations include cyanosis, respiratory distress, and hemodynamic instability within hours of delivery. Delays in diagnosis and delay in intervention will increase the morbidity of neonates with HLHS.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Purpose:&lt;/strong&gt; To understand the mechanism of HLHS along with the principles of anesthesia in neuroaesthetics procedures in pediatrics in order to obtain a good outcome.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Case Illustration:&lt;/strong&gt; A 9-day old baby girl, weight 2522 grams with HLHS (aortic atresia) with patent ductus arteriosus (PDA), type II atrial septal defect (ASD) with a left to right shunt, and dandy walker syndrome. From the physical examination, the patient's condition is stable, with GCS E4V5M6, pulse 130-135 beats/minute, breath rate 48x / minute, and SpO2 94-98%. The patient's head was enlarged from birth, no heart sounds were found, regular I-II heart sounds. Blood laboratory tests showed a total bilirubin value of 11.7 mg / dL, direct bilirubin 0.64 mg / dL, indirect bilirubin 10.43 mg / dL, hemoglobin 20.1 g%, hematocrit 55%, with leukocytes 19.9x103 / L, platelets 216 x 103 / L, serum Na 122 mmol / L, K 7.8 mmol / L, Cl 101 mmol / L.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Discussion:&lt;/strong&gt; The anesthetic approach in HLHS is to maintain preoperative hemodynamic stability by maintaining heart rate, preload, and PGE1, balancing systemic vascular resistance and pulmonary vascular resistance, preventing too high PaO2, and administering inotropic agents to increase cardiac output and keep the patent ductus arteriosus open. The balance of systemic and pulmonary blood flow is a key principle in the management of HLHS anesthesia. &lt;/p&gt;&lt;p&gt;&lt;strong&gt;Conclusion:&lt;/strong&gt; The main goal of HLHS anesthesia is to minimize hemodynamic changes to prevent compromised hemodynamics in both circulations and maintaining stability is essential in preventing morbidity, complications, and increasing good outcomes in surgery..&lt;/p&gt;&lt;p&gt; &lt;/p&gt;&lt;p&gt; &lt;/p&gt;

Pulmonary atresia with a ventricular septal defect and left pulmonary artery discontinuity: a case report\xa0

BackgroundUnilateral pulmonary artery discontinuity is a rare malformation that is associated with other intracardiac abnormalities. Cases accompanied by other cardiac abnormalities are often missed on prenatal echocardiography. The prenatal diagnosis of isolated unilateral pulmonary artery discontinuity can also be delayed. However, undiagnosed this malformation would have an effect on further prognosis. We report our case of a prenatal diagnosis of pulmonary atresia with ventricular septal defect and left pulmonary artery discontinuity.Case presentationA 33-year-old Asian woman visited our institution at 24 weeks of gestation because of suspected fetal congenital heart disease. Fetal echocardiography revealed a small atretic main pulmonary artery giving rise to the right pulmonary artery without bifurcation and the left pulmonary artery arising from the ductus arteriosus originating from the left subclavian artery. The neonate was delivered by cesarean section at 376/7 weeks of gestation. Postnatal echocardiography and multidetector computed tomography showed a right aortic arch, with the small right pulmonary artery originating from the atretic main pulmonary artery and the left pulmonary artery originating from the left subclavian artery. Patency of the ductus arteriosus from the left subclavian artery was maintained with prostaglandin E1. Right ventricular outflow tract reconstruction and pulmonary angioplasty with Gore-Tex graft patch was performed 25th day after birth. Unfortunately, the neonate died because of right heart failure 8 days postoperation.ConclusionThere is a possibility that both pulmonary arteries do not arise from the same great artery (main pulmonary artery or common arterial trunk). Therefore, clinicians should check the origin of both pulmonary arteries.

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.&#x0D; Keywords: congenital heart disease, duct-dependent circulation, ductus arteriosus, prostaglandin

Pulmonary Hypertension with Prolonged Patency of the Ductus Arteriosus in Preterm Infants.

There continues to be a reluctance to close the patent ductus arteriosus (PDA) in premature infants. The debate on whether the short-term outcomes translate to a difference in long-term benefits remains. This article intends to review the pulmonary vasculature changes that can occur with a chronic hemodynamically significant PDA in a preterm infant. It also explains the rationale and decision-making involved in a diagnostic cardiac catheterization and transcatheter PDA closure in these preterm infants.

Diagnosis and Management of Fetal Ductus Arteriosus Constriction

AbstractThe ductus arteriosus is an important structure in the fetal circulation. It connects the pulmonary artery to the arch of the aorta distal to the origin of the left subclavian artery and carries most of the right ventricular output. Patency of the ductus arteriosus is maintained by factors like prostaglandin E2, nitric oxide and low oxygen tension of the blood. With the advent of ultrasonography and Spectral Doppler, many cases of ductal constriction have been reported. It can be idiopathic but is usually secondary to the use of medication like NSAIDs, isoxsuprine, fluoxetine and also some particular foods rich in polyphenol like herbal teas, dark chocolate, berries and coffee. Idiopathic ductal constriction is a rare finding. Ductus arteriosus constriction occurs largely due to inhibition of the prostaglandin synthetase pathway as the ductus arteriosus becomes more sensitive to prostaglandin inhibitors in later gestation. It is, therefore, more commonly seen in the 3rd trimester. Because of fetal hyperdynamic compromise, it results in tricuspid regurgitation, right ventricular hypertrophy and ultimately right ventricular failure, hydrops, pulmonary hypertension and even intrauterine fetal death. We present a series of ductus arteriosus constriction, of which 3 cases were associated with tricuspid regurgitation, 1 case with right sided dominance of the heart and 1 case with moderate to severe right ventricular hypertrophy. Mild cases require close fetal monitoring and severe cases may need urgent delivery. Postnatal prognosis seems to be good in cases of ductal constriction associated with right heart abnormalities as right heart abnormalities resolve in a couple of weeks, although pulmonary hypertension and right ventricular failure are major concerns. Preventive measures include avoiding the use of NSAIDs and other medications especially in the 3rd trimester and changes in maternal dietary habits.

Tetralogy of Fallot: Origins, Management and Outcomes

Tetraology of Fallot (TOF) is the most common of the cyanotic congenital heart diseases. It is characterized by four anatomical lesions all of which have a spectrum of severity. Clinically the presentation of the neonate depends on the degree of Right Ventricular Outflow Tract (RVOT) obstruction and the patency of the ductus arteriosus with common symptoms including paroxysms of irritability, diaphoresis, hyper cyanotic spells and heart failure. Physicians currently use a multitude of investigations in order to demonstrate the complex physiology of TOF including sonography (in utero), Doppler echocardiography, cardiac catheterization, Multidetector CT (MDCT) and MRI. When used in combination these allow for planned surgical management of this otherwise life limiting condition which can be technically challenging due to variable anatomy. Currently patients should have curative surgery between 3-6 months with surgical approaches including VSD closure through a transaterial-transpulmonary approach and Transannular Patch (TAP) placement to limit RVOT. Currently, 97% TOF patients can expect to survive one year however they require very close follow up with cohort studies revealing high risks of ventricular arrhythmia and sudden cardiac death.

Metoda plastyki łuku aorty z zachowaniem przewodowego przepływu systemowego w leczeniu krytycznej koarktacji o morfologii bovine trunk

Introduction. Coarctation of the aorta is a congenital heart defect defined as aortic narrowing at the isthmus. It may occur in association with other defects, which requires a discussion regarding the choice of optimal therapy. Critical aortic coarctation with aortic hypoplasia and so-called bovine trunk anatomy is one of the more challenging forms of this defect, characterized by a common origin of the brachiocephalic trunk and the left common carotid artery, which form a common vessel extending as a direct continuation of the ascending aorta. Therapeutic decisions are made depending on the distance between the ascending aorta and the isthmus and descending aorta, taking into account the typical management of hypoplastic aortic arch. Material and methods. The clinical presentation in the neonatal period includes weak or absent femoral pulse, pallor, apathy, dyspnea and signs of systemic hypoperfusion with progressive lung edema and acidosis. The diagnosis is mostly based on echocardiography while magnetic resonance imaging or computed tomography allow detailed imaging of the collateral vessels. The most important diagnostic issues are the anatomy of the defect, severity of concomitant aortic isthmus narrowing, and its hemodynamic effect. The medical management is directed at maintaining patency of the ductus arteriosus with prostaglandin E1 infusion and an appropriate balance between peripheral systemic and pulmonary resistance. The recommended treatment of choice is an early surgical correction of the aortic arch defect. The aortic arch reconstruction method with ductus-dependent systemic flow preservation implies resection of the coarctation along with the isthmus narrowing and proximal end-to-side anastomosis, with reconstruction of the inflow to the left subclavian artery using a hypoplastic distal segment of the aortic arch. The operation is performed by the left posterolateral thoracotomy without using extracorporeal circulation. Conclusions. The prerequisite for selecting a neonate for the proposed procedure is a detailed assessment of aortic arch anatomy, with particular attention to the measurement of the distance between the ascending aorta and the area where the ductus arteriosus connects to the descending aorta. The major advantage of this technique is the ability to perform an off-pump procedure via the lateral access, using only healthy native patient tissues for aortic flow restoration.