Pulmonary Hypertensive Crisis Research Articles

Extracorporeal membrane oxygenation (ECMO) support for children with pulmonary hypertension: A single-institutional experience of outcomes.

Pediatric pulmonary arterial hypertension (PAH) can present with a wide spectrum of disease severity. Pulmonary hypertension (PH) crises can lead to acute decompensation requiring extracorporeal membrane oxygenation (ECMO) support, including extracorporeal cardiopulmonary resuscitation (eCPR). We evaluated outcomes for pediatric PH patients requiring ECMO. A single-institution retrospective review of pediatric PAH patients with World Symposium on PH (WSPH) groups 1 and 3 requiring ECMO cannulation from 2010 through 2022 (n = 20) was performed. Primary outcome was survival to hospital discharge. Secondary outcomes were survival to decannulation and 1-year survival. Of 20 ECMO patients, 16 (80%) survived to decannulation and 8 (40%) survived to discharge and 1 year follow up. Of three patients who had two ECMO runs; none survived. There were five patients who had eCPR for the first run; one survived to discharge. The univariate logistic regression model showed that venovenous ECMO was associated with better survival to hospital discharge than venoarterial ECMO, (OR: 0.12, 95% CI: 0.01-0.86, p = 0.046). PH medications (administered before, during, or after ECMO) were not associated with survival to discharge. For children with decompensated PAH requiring ECMO, mortality rate is high, and management is challenging. While VA ECMO is the main configuration for decompensated PH, VV ECMO could be considered if there is adequate ventricular function, presence of a systemic to pulmonary shunt, or an intercurrent treatable illness to improve survival to discharge. A multidisciplinary approach with requisite expertise should be utilized on a case-by-case basis until more reliable data is available to predict outcomes.

Screening and Perioperative Management of Obesity Hypoventilation Syndrome.

Obesity hypoventilation syndrome (OHS) can often be underdiagnosed or misdiagnosed and has been shown to pose significant risks in perioperative situations. Patients with OHS have a higher prevalence of baseline morbid conditions like hypertension, congestive heart failure (CHF), diabetes mellitus, atrial fibrillation, and pulmonary hypertension (PH), which contribute to adverse postoperative outcomes. The potential challenges include difficult intubation and loss of airway, postoperative respiratory failure, worsening heart failure, pulmonary hypertensive crisis, and opioid-induced respiratory depression (OIRD). It is, therefore, important to screen all obese patients for obstructive sleep apnea (OSA) and OHS before elective surgical procedures. The aim of this review is to discuss the preoperative screening and evaluation and safe anesthetic and up-to-date ventilatory management of this complex group of patients. This review also intends to increase the awareness of OHS in the adult population among hospitalists, surgeons, and cardiologists who may find themselves taking care of these patients in complex multidisciplinary settings.

Comparative Analysis of Treatment Strategies for Postoperative Pulmonary Hypertensive Crisis in Congenital Heart Disease

Background: Pulmonary hypertensive crisis (PHC) is a serious life-threatening complication in children with congenital heart disease (CHD) after surgery, with acute onset and high mortality. However, there is still no effective means to deal with this complication. Therefore, our department developed the left atrium and right ventricle duct bridging technique to treat children with PHC, and compare its effects with conventional methods of treatment to determine the best solution for dealing with this complication. Methods: A retrospective analysis of 41 children with CHD surgery and postoperative PHC in our hospital from January 2015 to December 2022 was performed. According to the rescue method, the group with conventional therapy combined with left atrium to right ventricle duct bridging treatment vs. simple conventional therapy were defined as group A and group B respectively, with 13 cases and 28 cases in each group. The success rate of rescue, the complication rate, the length of intensive care unit stay, the delayed chest closure duration, the duration of PHC, the ratio of pulmonary circulation/systemic circulation pressure, the oxygenation index and the cardiac index at 30 min, 1 h, 2 h, 4 h and 6 h after rescue were compared between the two groups. Results: There was a higher success rate of rescue (84.62% vs. 68.86%; p > 0.05), lower complication rate (15.38% vs. 21.43%; p > 0.05), shorter duration of PHC (6.77 ± 2.13 min vs. 13.07 ± 4.05 min; p = 0.000), shorter duration of delayed sternal closure (32.23 ± 5.46 h vs. 38.14 ± 8.61 h; p = 0.029) and shorter length of ICU stay (81.69 ± 8.31 h vs. 93.57 ± 16.84 h; p = 0.021) in group A; After the rescue, the pulmonary artery pressure and cardiopulmonary function recovery rate in group A were faster than those in group B. Conclusions: In conclusion, left atrial to right ventricular duct bridging therapy is more effective in the treatment of PHC after surgery for CHD.

Paediatric pulmonary hypertension due to congenital heart disease in non-cardiac surgery: Anaesthetic implications

Paediatric patients with pulmonary hypertension associated with Congenital Heart Disease (PH-CHD) are frequently encountered in non-cardiac surgical settings. Although the pharmacological and surgical management of the underlying CHD in the group has improved yet its management remains challenging due to associated high morbidity and mortality. The dilemma prevails more so because of the dearth of adequate literature describing its pathophysiology and management in non-cardiac surgical settings. There are no specific guidelines pertaining to paediatric PH-CHD care, and the precepts have been extrapolated from adult studies and guidelines. This review article intends to apprise the pathophysiology of PH-CHD, its management, and its perioperative care with special emphasis on pulmonary vascular hypertensive crisis in these patients posted for non-cardiac procedures.

Impact of long lasting pulmonary arterial hypertension on the surgery of congenital heart defect

BackgroundLittle is known about the management of this high-risk subgroup of patients because only a few small retrospective studies were available in the literature. ObjectivesThis study was designed to identify the determinants of post operative mortality and to assess during 10 years follow-up functional and hemodynamic variables for children presenting at surgery with pulmonary vascular disease associated with congenital heart defect. MethodsThis is a prospective study that enrolled children with pulmonary arterial hypertension associated with congenital heart disease and presenting beyond the optimal time frame for surgery. Inclusion criteria were late presentation for surgery and severe pulmonary arterial hypertension, defined as mean pulmonary arterial pressure > 50 mmhg. An univariable analysis, receiver operating characteristic curves, and Kaplan-Meier curves were used for survival analysis. Results75 patients underwent repair of their cardiac lesions. The operative mortality rate was 16%(12 patients); 43 patients (57 %) had right heart catheterization, and all of them were positive for acute vasodilatatory challenge (NO, oxygen). In the postoperative period, 32 % of patients had pulmonary hypertensive crisis, and 72 % had right heart failure. During follow-up, pulmonary arterial hypertension subclass 4 was present in 5% of our patients. ConclusionOur study indicated that for operable patients but beyond the optimal time frame the surgical mortality remained high despite using iNO. However, for those who survived they achieved good results in functional exercise, their PAP dropped to normal level and small proportion had persistent pulmonary arterial hypertension encouraging surgical closure of the defect.

How to Recognize and Overcome Pulmonary Hypertension Crisis During Patent Ductus Arteriosus Closure by Device in Adult

Background: Managing a patent ductus arteriosus (PDA) with severe pulmonary hypertension (PH) is challenging since closing the PDA can result in reduced cardiac output and right ventricular (RV) failure. The latest guideline for Adult Congenital Heart Disease (ACHD) stated it is harmful to close the defect in patients with pulmonary vascular resistance (PVR) ≤5 WU and flow ratio (FR) <1.5. Case presentation: A 37-year-old female was referred with cough, fever, low saturation, and murmur findings. After serial examination, she was diagnosed with large PDA and severe PH her peripheral saturation (SaO2) was 88%-89% and non-responder to acute vasoreactivity test. After a year of PH therapy, vasoreactivity showed a response to the vasoreactivity test and improved on clinical presentation with SaO2 91%-92%. Patient fell in to PH crisis condition during the procedure of device closure, prostacyclin analogue intravenous (IV) and phosphodiesterase inhibitors inhalation was administered, and the procedure was carried on. The device was successfully implanted, and the patient had SaO2 97% in all four extremities before discharge. Conclusion: With established PH therapy, PDA with severe PH can underwent PDA closure by device with satisfying outcome. Keyword: Case Report, Congenital Heart Disease Intervention, Patent Ductus Arteriosus, Pulmonary Hypertension Crisis.

Late Presentation of Pulmonary Hypertension Crisis Concurrent with Atrial Arrhythmia after Atrial Septal Defect Device Closure

Background: ASD occurs when there is a septal defect between the right and left atria, resulting in a left-to-right shunt that increases the volume of the right heart and pulmonary circulation. Increased pulmonary resistance can lead to pulmonary hypertension (PH), resulting in progressive deterioration of right ventricular function, leading to right heart failure and death. Prolonged elevation of atrial pressure induces progressive atrial dilatation and electrophysiological remodelling. Together with autonomic modulation, this leads to atrial arrhythmias (AAs). Patients with significant shunts leading to ventricular volume overload are considered for ASD closure. However, in some cases, PH occurs after ASD closure.  Case Presentation: We report a 21 yo man diagnosed with ASD Secundum Post Closure with Device (September 1th, 2023) and Pulmonary Hypertension Crisis. The left atria (LA), right atria (RA) and right ventricle (RV) were dilated. We also found moderate mitral regurgitation, severe tricuspid regurgitation, and mild to moderate pulmonary regurgitation. There was a decline in systolic function in the right ventricle, and grade III diastolic dysfunction in left ventricle. There was a well-seated device with no residual shunt on interatrial septal. The pulmonary arteries were confluence and dilated. From ECG we found atrial flutter with variable conduction. This patient was transferred to HCU. This patient treated with digoxin, furosemide, milrinon, ceftriaxone, miniaspi, sildenafil, electrophysiology, and 3D ablation.  Conclusion: Pulmonary hypertension can occur in cases of congenital heart defects, such as ASD. The operative management of ASD is closure of the ASD, but in some unique groups, this can lead to pulmonary hypertensive crisis after its closure.

Percutaneous Secundum Atrial Septal Defect Closure: Failure Rate and Procedural Predictors

Background: Percutaneous atrial septal defect (ASD) closure is one of therapeutic options for patients with a suitable ASD anatomy, however in developing countries, the exact figure and procedural characteristics remain unknown. Therefore, this study was conducted to identify the failure rate and procedural predictors of the percutaneous ASD closure.Materials and methods: A retrospective study using a database of all patients undergoing percutaneous ASD closure was conducted between March 2010 to November 2023. Patients who developed a pulmonary hypertensive crisis during the procedure were excluded. Procedural and echocardiographic parameter were measured and analyzed.Results: A total of 112 samples were included in this study, 74.1% were female and 55.36% were pediatric patients. The failure rate was 12.5% (n=14) with diameter index was higher in the failed group. Unpaired T-test revealed that ASD size was one of the predictor failure in pediatric patients (mean diameter: 24.7±6.46 mm vs. 16.36±5.94 mm, p=0.001). There were no statistically significant variations in rim diameters, while compared with all patients with appropriate rims (rim ≥7mm), the failure rate was higher in patients with two rims measuring between 5.9 and 6.9 mm and rims less than 5 mm. Two patients presented with device embolization and required surgical device removal.Conclusion: The failure rate of percutaneous ASD closure was 12.5%. A larger ASD increases the risk of failure of percutaneous closure in pediatric patients. Furthermore, patient with 5-6.9 mm on two or more rims as well as those with rim less than 5 mm, have a higher failure rate.Keywords: secundum atrial septal defect, percutaneous closure, failure rate

Successful Late Repair of Truncus Arteriosus: A Single Center Experience.

Background: Truncus arteriosus is a rare congenital heart defect resulting from the failure of the truncus arteriosus to divide during fetal development. It leads to a single outflow tract from the heart and, if left untreated, can be fatal. Late presentation and repair can also increase the risk of pulmonary hypertensive crises, which can lead to morbidity and mortality after repair. Methods: We performed a retrospective study examining outcomes of late-presenting patients who were repaired for this anomaly at our institution. Results: We identified seven patients who underwent late repair of truncus arteriosus who were 3 to 11 years of age. There were six females and one male. Postoperatively, all patients showed improvement in symptoms and hemodynamic parameters, with no reported mortality. The median duration of stay in the intensive care unit was nine days and with a range from 3 to 18 days, while the median hospital stay was 29 days with a range from 21 to 60 days. Conclusion: These findings highlight the potential for successful outcomes even in cases of delayed diagnosis.

Reversible severe pulmonary hypertension related to scurvy in children

BackgroundSevere pulmonary hypertension (PH) in childhood is rare and can manifest as a life-threatening episode. We present 2 children with restrictive dietary habits with severe pulmonary hypertension secondary to scurvy and iron deficiency anemia with treatment and outcome.Case presentationThe first case is a 2-year-old boy who presented with vomiting, diarrhea, and fever. After rehydration, he had recurrent episodes of hypotension with intermittent abdominal pain. Fluid resuscitation and inotropic medication were given. Then he suddenly collapsed. After 4-min cardiopulmonary resuscitation, his hemodynamic was stabilized. Most of the medical workup was unremarkable except for PH from the echocardiogram with estimated systolic pulmonary artery pressure (PAP) at 67 mmHg. Transient PH was diagnosed, and milrinone was prescribed. Since he had restrictive dietary habits and sclerotic rim at epiphysis in chest films, his vitamin C level was tested and reported low-level result. The second case is a 6-year-old boy with acute dyspnea, a month of low-grade fever, mild cyanosis, and a swollen left knee. Echocardiogram indicated moderate TR with estimated systolic PAP at 56 mmHg (systolic blood pressure 90 mmHg). Milrinone was given. Right cardiac catheterization showed PAP 66/38 (mean 50) mmHg and PVRi 5.7 WU.m2. Other medical conditions causing PH were excluded. With a history of improper dietary intake and clinical suspicion of scurvy, vitamin C was tested and reported undetectable level. Administration of vitamin C in both cases rapidly reversed pulmonary hypertension.ConclusionPediatric PH related to vitamin C deficiency can manifest with a wide range of symptoms, varying from mild and nonspecific to severe life-threatening episodes characterized by pulmonary hypertensive crises. PH associated with scurvy is entirely reversible with appropriate investigation, diagnosis, and treatment. Our report highlights the importance of considering nutritional deficiencies as potential confounding factors in pediatric PH, emphasizing the need for comprehensive evaluation and management of these patients.

Identification of proinflammatory pathways and promising bioactive polyphenols for the treatment of sickle cell anemia by in silico study and network pharmacology

Sickle cell anemia (SCA) is an autosomal recessive Mendelian trait characterized by symptoms that include acute and chronic pain, chest syndrome, pulmonary hypertension, stroke, kidney disease, and vaso-occlusive crises (VOCs), all of which worsen with age; VOCs are the leading cause of hospitalization and premature death in SCA patients. Currently, despite the existence of treatments for SCA, the negative consequences of VOCs’ chronic inflammatory state demand the exploration of alternative methods of control. For this reason, the goal of this research was to find novel pathways and promising bioactive polyphenols for the treatment of SCA using a combination of network pharmacology and in silico approaches; due to polyphenols, they have shown widely reported anti-inflammatory properties. Initially, hub genes associated with inflammatory processes in SCA were identified by extracting differentially expressed genes (DEGs) from a publicly available GEO dataset (GSE53441), followed by their validation through system biology analysis, Polyphenols with anti-inflammatory activity were selected from natural product databases; finally, molecular docking and dynamics were performed with the polyphenols and the key protein derived from the selected hub genes. As a result, 10 genes associated with the Type I interferon (IFN–I) pathway in SCA were identified (MX1, FIT1, IFIT3, STAT1, ISG15, GBP1, OAS1, OAS2, OAS3, and RSAD); among them, STAT1 was selected as a central hub gene by regulating the expression of the rest. Docking and dynamics studies showed good binding energies among STAT1 and the fifteen polyphenolic extracted compounds, with quercetin, diosmetin, and fisetin showing the lowest binding energies. Identified flavonoids have been described in the past as compounds having anti-inflammatory and antioxidant features, as well as possible alternatives for SCA treatment.

Evaluation of maternal-fetal outcomes in pregnancy complicated with severe pulmonary hypertension and its influencing factors: a single-center retrospective study in China

ObjectivePregnancy is not recommended for patients with severe pulmonary hypertension (PH) due to the significant risks it poses to both the mother and fetus. The objective of this study is to describe the maternal-fetal outcomes in pregnant women with PH and identify the factors that influence these outcomes.MethodThis retrospective study analyzed clinical data from 25 patients with severe PH who were admitted to our hospital between January 2018 and December 2022. The data we used came from a public general hospital in Fujian Province.ResultsThe mean systolic pulmonary artery pressure (sPAP) of 25 patients was 105.12 ± 22.70 mmHg. All patients had received one or more multidisciplinary team (MDT) treatments before terminating their pregnancies. Among the pregnant women, four experienced a pulmonary hypertensive crisis (PHC), seven had heart failure, and one had postpartum hemorrhage (PPH). Among them, seven (28%) pregnant women died primarily due to heart failure and PHC. Among the fetal outcomes, twelve resulted in therapeutic abortion, and eleven resulted in preterm birth. Among the perinatal complications, eleven infants (84.6%) were born prematurely, six infants (46.2%) experienced neonatal asphyxia, eight infants (61.5%) had low birth weight, and two infants (15.4%) died during the perinatal period. According to the etiology, seven individuals had idiopathic pulmonary arterial hypertension (iPAH), ten had pulmonary arterial hypertension associated with congenital heart disease (CHD-PAH), six had pulmonary hypertension associated with left heart disease (LDH-PH), and two had pulmonary arterial hypertension caused by other diseases (oPAH). The sPAP levels of iPAH and CHD-PAH were significantly higher than those of LDH-PH and oPAH (p < 0.05). Additionally, the gestational weeks of LDH-PH were higher than those of iPAH (p < 0.05). The number of patients with New York Heart Association (NYHA) heart function grade III-V was higher in the death group compared to the non-death group (p < 0.05).ConclusionPregnancy in women with severe PH carries a high risk of mortality. Therefore, contraception is strongly recommended for these women. NYHA cardiac function grade III-IV was useful in predicting the risk of mortality.

Long-Term Erythrocytapheresis Did Not Prevent Development of Severe Pulmonary Hypertension in Sickle Cell Disease

Background The WHO classifies pulmonary hypertension into five different groups based on pathophysiology, clinical presentation, and therapeutic options. The development of pulmonary hypertension (PH) in sickle cell disease (SCD) is multifactorial and can cause devastating complications. Erythrocytapheresis is utilized in patients with SCD who have multiple comorbidities or are unresponsive to first-line treatments like hydroxyurea. It is hypothesized that treatment with long-term erythrocytapheresis (LTE) could slow the progression to pulmonary hypertension by modifying the pathophysiology of the disease process. However, limited clinical data supports this transfusion modality [1]. Case Description The patient is a 17-year-old female with a history of SCD, obstructive sleep apnea (OSA), nocturnal hypoxemia, and recurrent pain crises maintained on LTE every four weeks for the past seven years. She had previously failed hydroxyurea therapy. She underwent a tonsillectomy and adenoidectomy seven years ago to treat OSA. The patient had an echocardiogram five years ago, which showed normal biventricular function and tricuspid regurgitation jet velocity. Recently she presented to the emergency department with cough, shortness of breath, fever, diarrhea, and nausea lasting for three days. Initial assessment revealed elevated liver enzymes, elevated BNP, hyperbilirubinemia, hemoglobin of 9.5 g/dL, and oxygen saturations in the 80's. Echo revealed right ventricular dysfunction and right atrial and ventricular enlargement with a tricuspid regurgitation jet velocity of 4.29 m/s and estimated right ventricular systolic pressure 90% systemic, as seen in figure 1. The patient was admitted to the PICU under concern of pulmonary hypertension crisis. She underwent a right cardiac catheterization three days into her admission and was diagnosed with severe pulmonary hypertension with a mean pulmonary artery pressure of 49 mmHg. Pulmonary capillary wedge pressure was 11 mmHg, and pulmonary vascular resistance was calculated to be 18 wood units x m 2 with 20 PPM of nitric oxide and 80% FiO2 the PVR decreased to 11 woods units x m 2 (figure 2). CTA showed subsegmental thrombosis and vascular surgery was consulted for evaluation for endarterectomy, but they decided she was not a surgical candidate. She was initiated on anticoagulation therapy with enoxaparin, sildenafil, and bosentan. Conclusions The cause of PH in this patient appears to be multifactorial. Possible etiologies include SCD-related, primary pulmonary hypertension, or subsegmental pulmonary embolism. Although LTE is increasingly used in the management of SCD, providers should not assume that a patient receiving this treatment will not progress to develop complications. Some patients on LTE may also develop progressive CNS vasculopathy and are still at risk for developing hypertension. Patients with SCD should be screened for PH if they are symptomatic or have comorbid conditions. This patient had a history of nocturnal hypoxemia and OSA, which some studies indicate are associated with pulmonary hypertension in adolescents with SCD. Screening for nocturnal hypoxemia and OSA is recommended in SCD to identify patients at high risk of PH development [2].

Supracardiac Total Anomalous Pulmonary Venous Connection in Adolescence.

Background Supracardiac total anomalous pulmonary communication (TAPVC) constitutes a rare congenital cardiac anomaly. Most babies with supracardiac TAPVC are diagnosed in infancy and undergo complete surgical repair during infancy. Delayed presentation of supracardiac TAPVC is rare, and the surgical outcomes are not well known. This retrospective study was conducted to determine the presentation and surgical outcome of supracardiac TAPVC among adolescents, which constitutes an extremely rare subgroup of TAPVC. Methodology This retrospective analysis was conducted among 15 adolescent patients with supracardiac TAPVC who underwent surgical repair in the cardiothoracic surgery department of a tertiary care center in India. This study aimed to assess the intraoperative, postoperative, immediate, early, and late outcomes of adolescent patients with supracardiac TAPVC who had undergone surgical repair between 2010 and 2014 in a tertiary care center in India. Results The study included 15 patients with a diagnosis of isolated supracardiac TAPVC. A mild degree of cyanosis was present in eight patients, recurrent episodes of lower respiratory tract infections were present in five patients, and dyspnea (New York Heart Association I/II) was noted in 12 patients. Mean oxygen saturation was 92% (range = 85-93%), and mean pulmonary artery pressure was 24 mmHg (range = 15-50 mmHg). After median stenotomy, a wide anastomosis was made between the common pulmonary venous chamber and the posterior wall of the left atrium. A fenestration was made in the Dacron patch in three patients who had raised pulmonary vascular resistance (PVR) preoperatively. Twelve patients were weaned off cardiopulmonary bypass (CPB) with minimal inotropic support. Three patients who had high preoperative PVR had difficulty in weaning from CPB. The mean CPB and cross-clamp time was 75 ± 12 minutes and 58 ± 9 minutes, respectively. Atrial fibrillation was noted in five (33.3%) patients in the early postoperative period, and three (20%) patients had pulmonary artery hypertensive crises postoperatively. There was no superficial or deep sternal wound infection in the postoperative period. Mild and moderate right ventricular dysfunction was present in four (26.67%) and two (13.3%) patients, respectively, in the postoperative period. On two-dimensional echocardiography during follow-up at the end of one year, there was no gradient across the anastomosis, and pulmonary artery pressure was normal in all patients. Conclusions Surgical repair of supracardiac TAPVC in adolescence has an excellent outcome. Survival of patients with supracardiac TAPVC until adolescence depends on the presence of a dilated vertical vein and a large atrial septal defect facilitating unobstructed pulmonary venous flow. The aim of the surgical repair should be to create a wide anastomosis between the left atrium and the pulmonary venous chamber which should be bigger than the size of the mitral valve orifice indexed to the body surface area as it would amount to no or negligible anastomotic gradient postoperatively.

Preoperative Sildenafil in Pediatric Patients Undergoing Congenital Heart Surgeries: A Systematic Review and Meta-Analysis.

Background. Pulmonary hypertension (PH) is a common complication of congenital heart disease (CHD). With early surgical intervention, outcomes have improved over the last two decades. Persistent PH, however, may still occur following surgery. Sildenafil has been shown to be beneficial for postoperative pulmonary hypertensive crises. The role of preoperative sildenafil in controlling postoperative PH is poorly elucidated. This study aimed to evaluate the effect of preoperative sildenafil on pediatric patients undergoing congenital heart surgeries. Methods. A comprehensive literature search was conducted in scientific databases. We included randomized controlled trials which assessed the effect of preoperative sildenafil in pediatric patients with CHD undergoing repair surgeries. Primary outcomes were pre- and postoperative differences in mean pulmonary arterial pressure (mPAP) and mean pulmonary artery/aortic pressure ratio (PA/Ao ratio). Results. Four studies (n = 233) were retained for the final analysis. Dose of sildenafil ranged from .3 to .5mg/kg every 4-6hours via oral/nasogastric route, with timing of administration varied from 1 to 2weeks before surgery. Compared to controls, preoperative sildenafil was associated with greater reduction in postoperative mPAP (MD -5.02; 95% CI [-8.91, -1.13]) and mean PA/Ao ratio (MD -.11; 95% CI [-.17, -.06]). Shorter CPB time, ICU length of stay, and duration of mechanical ventilation were also observed in the sildenafil group. Conclusion. Preoperative sildenafil is beneficial in reducing PAP, thereby reducing the risk of postoperative PH crisis. Further studies are warranted to identify the optimal dosage and timing of administration of sildenafil in CHD patients prior to surgery.

Pulmonary Hypertension in Pregnancy: A Case Report

Background: Pulmonary Hypertension (PH) is a disease characterized by distressing symptoms and decreased life expectancy due to the narrowing of the blood vessels of the lungs, which often leads to right heart failure. The prevalence of PH in women is 97 cases per million, with 64% of the main causes of PH in pregnancy congenital heart defects, resulting in a very high maternal and fetal mortality rate. Case Report: A 38-year-old G4P1A2 31-week gestational age complained of shortness of breath for 5 days. Physical examination revealed blood pressure was 107/62 mmHg, Heart Rate was 98 beats per minute, respiration 40x per minute, and SpO2 88% with NRM 10 Lpm. The heart examination obtained heart sound I -II regular and a systolic murmur was heard between the left 2nd ribs. Abdominal examination was single fetus, intrauterine, breech presentation, His (+), fetal heart rate 160 beats per minute. vaginal toucher 2 cm in labour. ultrasound examination singles fetal, transverse lies, with an estimated fetal weight of 1600 grams. Echocardiography finding: ASD II L to R shunt with LV EF 60%(T), 62% (S), dilated RA-RV, TR severe, MR mild, High Probability of Pulmonary Hypertension. The patient was diagnosed with Dyspnea, Pulmonary edema caused by cardiogenic, ASD II, High probability of PH, NYHA IV, and Breech presentation in labor. Decided to perform a caesarian section and sterilization. The Male baby was born with 1570 grams Apgar Score 3-5-7. post operation patient was admitted to ICU. Twelve hours after the operation the patient had decreased control and became a PH crisis then the patient was declared dead. Results: The death of the patient, in this case, was caused by cardiogenic shock due to Pulmonary Hypertension Crisis. Conclusion: Early diagnosis along with collaborative and comprehensive management of pulmonary hypertension is needed for good maternal and fetal outcomes. Keywords: pulmonary hypertension, pregnancy, heart disease. Correspondence: Trisulo Wasyanto. Department of Cardiology and Vascular Medicine, Faculty of Medicine, Sebelas Maret University / Dr. Moewardi Hospital. Jl. Kol. Sutarto 132, Surakarta 57126, Central Java, Indonesia. Email: trisulo.wasyanto@staff.uns.ac.id. Mobile: +62811294225.

Anesthetic management of children with medically refractory pulmonary hypertension undergoing surgical Potts shunt.

Pulmonary hypertension in children is associated with high rates of adverse events under anesthesia. In children who have failed medical therapy, a posttricuspid shunt such as a Potts shunt can offload the right ventricle and possibly delay or replace the need for lung transplantation. Intraoperative management of this procedure, during which an anastomosis between the pulmonary artery and the descending aorta is created, is complex and requires a deep understanding of the pathophysiology of acute and chronic right ventricular failure. This retrospective case review describes the intraoperative management of children undergoing surgical creation of a Potts shunt at a single center. A retrospective case review of all patients under the age of 18 who underwent Potts shunt between April 2013 and June 2022. Medical records were examined, and clinical data of demographics, intraoperative vital signs, anesthetic management, and postoperative outcomes were extracted. Twenty-nine children with medically refractory pulmonary hypertension underwent surgical Potts shunts with a median age of 12 years (range 4 months to 17.4 years). Nineteen Potts shunts (65%) were placed via thoracotomy and 10 (35%) were placed via median sternotomy with use of cardiopulmonary bypass. Ketamine was the most frequently utilized induction agent (17 out of 29, 59%), and the majority of patients were initiated on vasopressin prior to intubation (20 out of 29, 69%). Additional inotropic support with epinephrine (45%), milrinone (28%), norepinephrine (17%), and dobutamine (14%) was used prior to shunt placement. Following opening of the Potts shunt, hemodynamic support was continued with vasopressin (66%), epinephrine (62%), milrinone (59%), dobutamine (14%), and norepinephrine (10%). Major intraoperative complications included severe hypoxemia (21 out of 29, 72%) and hypotension requiring boluses of epinephrine (10 out of 29, 34.5%) but no patient suffered intraoperative cardiac arrest. There were four in-hospital mortalities. A Potts shunt offers another palliative option for children with medically refractory pulmonary hypertension. General anesthesia in these children carries high risk for pulmonary hypertensive crises. Anesthesiologists must understand underlying physiological mechanisms responsble for acute hemodynaic decompensation during acute pulmonary hypertneisve crises. Severe physiological perturbations imposed by thoracic surgery and use of cardiopulmonay bypass can be mitigated by aggresive heodynamic support of ventricle function and maintainence of systemic vascular resistance. Early use of vasopressin, before or immidiately after anesthesia induction, in combination with other inotropes is a useful agent during the perioperative care of thes. Early use of vasopressin during anesthesia induction, and aggressive inotropic support of right ventricular function can help mitigate effects of induction and intubation, single-lung ventilation, and cardiopulmonary bypass. Our single center expereince shows that the Potts shunt surgery, despite high short-term mortaility, may offer another option for palliation in children with medically refractory pulmonary hypertension.

Surgical repair of neonatal total anomalous pulmonary venous connection: A single institutional experience with 241 cases

ObjectiveChallenges persist in surgery for neonatal total anomalous pulmonary venous connection (neoTAPVC), with the high mortality risk not mitigated over time. MethodsA prospectively collected single-center database containing all neonates with TAPVC undergoing biventricular repair in 2012 to 2020 was retrospectively reviewed. The primary outcome was death or postoperative pulmonary venous obstruction (PPVO). Based on the preoperative admission location in our hospital, patients were classified into those being admitted to cardiac intensive care unit versus neonatal intensive care unit or general pediatric intensive care unit. Access to dedicated presurgical care (DPC) was defined as patients who were preoperatively admitted to the cardiac intensive care unit. ResultsOverall, 241 patients with a median age at surgery of 14 days (interquartile range [IQR], 9-21 days) were included. Anomalous return was supracardiac in 38.6%, cardiac in 26.1%, infracardiac in 28.6%, and mixed in 6.6%. Patients receiving DPC had better survival (96.3% vs 84.3%; P = .0028) and lower incidence of PPVO (15.2% vs 28.6%; P = .011) compared with those without DPC. Patients in the DPC group were less likely to undergo operation within 24 hours on presentation (27.1% vs 40.3%; P = .041), had improved lactate clearance (1.5 [IQR, 1.0-2.2] vs 2.8 [IQR, 1.8-4.1]; P < .001), and had lower incidence of postoperative pulmonary hypertension crisis (2.8% vs 18.7%; P < .001) compared with those in no-DPC group. After matching, no difference in PPVO could be observed in patients undergoing conventional versus sutureless repair (22.6% vs 12.9%; P = .29). ConclusionsAccess to DPC potentially improves outcomes in the neoTAPVC setting; freedom from PPVO were similar using conventional versus sutureless repair.

Surgical outcomes of common arterial trunk repair beyond infancy.

The aim of this study is to analyze the clinical outcomes of common arterial trunk repair beyond infancy in terms of both early- and long-term outcomes. Between January 2003 and December 2019, 56 patients underwent repair for common arterial trunk beyond infancy at our institute. Median age was 34.5months, 51.8% were females, and 48.2% were males. 48.2% were type 1, 46.4% were type 2, and 5.4% were type 3. 17.9% patients underwent direct connection technique for right ventricular outflow tract reconstruction; remaining received a conduit. The most common type of truncal valve anatomy was tricuspid (82.1%). Early mortality was 7%. Univariable analysis identified age (p = 0.003), weight (p = 0.04), duration of ventilation (p = 0.036), and pulmonary hypertensive crisis (p ≤ 0.001) as factors affecting early mortality. In our overall cohort of beyond infancy repair for common arterial trunk, at 10years, the survival, freedom from reintervention for right ventricular outflow tract reconstruction, freedom from ≥ moderate conduit obstruction, freedom from impaired right ventricle function, and freedom from ≥ moderate truncal valve regurgitation were 76.7%, 89.7%, 74%, 88.6%, and 66.3%, respectively. Repair for common arterial trunk in patients presenting beyond 1year of age is challenging; however, it can be done with satisfactory early and late outcomes in terms of mortality and reintervention.

Anesthetic Management of an Unusual Case of Severe Pulmonary Hypertensive

The perioperative management of pulmonary hypertension continues to be a great challenge for anesthesiologists. Among the various types of pulmonary hypertension, pulmonary hypertension associated with left heart disease is the most common, while pulmonary hypertensive crisis is one of the causes of perioperative mortality in these patients. We present the case of a 54-year-old female with severe valvular heart disease complicated by severe pulmonary hypertension and massive hemothorax, who underwent emergency exploratory thoracotomy, mitral valve replacement, and tricuspid valvuloplasty. The patient was in critical condition and suspected to have a pulmonary hypertension crisis. The rationale for this case report is to learn about the anesthetic management of patients with severe pulmonary hypertension.