Pulmonary Hypertensive Crisis Research Articles

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.

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.

Erectile Dysfunction in a Male Patient With Limited Cutaneous Systemic Sclerosis

A 24-year-old male with a 3-year history of limited cutaneous systemic sclerosis (LcSS) was referred to the urology clinic by his rheumatologist because of erectile dysfunction (ED). He complained of his worsening ED over the past two years. His international index of erectile function (IIEF) score was 5, indicating severe ED. He reported no morning erection and experienced some degree of turgidity without rigidity in sexual occasions. Digital ulcers and gastroesophageal reflux were his only presentations other than ED. His history was negative for pulmonary hypertension and renal crisis.

Hematopoietic stem cell transplantation, a curative approach in infantile osteopetrosis.

Most patients with osteopetrosis (OPT) can be causally and curatively treated with allogeneic hematopoietic stem cell transplantation (HSCT) because osteoclasts are derived from the HSC. However, HSCT is contraindicated in some forms of OPT, namely OPT with neurodegeneration (in all patients with OSTM1 and about half of patients with CLCN7 mutations) and OPT caused by an osteoblast defect (patients with RANKL mutations). HSCT for OPT risks serious side effects, such as transplant failure, venous occlusive disease, pulmonary hypertension, and hypercalcemic crises. Nevertheless, the success rate of HSCT has improved significantly in recent decades. This applies, in particular, to HSCT from non-HLA compatible (haploidentical) donors. Therefore, nowadays an HSCT can be discussed for intermediate OPT forms. After a successful HSCT, most patients have very good quality of life, but about two-thirds are visually impaired, and in rarer cases show motor and neurological disabilities. Early diagnosis, further improvements in transplantation procedures, and advances to improve quality-of-life after transplantation are challenges for the future.

Atrial septostomy to prevent pulmonary hypertension crisis in children with ventricular septal defect (VSD) and pulmonary hypertension (PH) underwent cardiac surgery: a case series

Severe pulmonary hypertension (PH) was reported in 22.4% of ventricular septal defect (VSD) and it was mainly seen on a large VSD. Atrial septostomy (AS) could improve the hemodynamic condition and long-term survival of PH patients. Here, three VSD and PH cases in children who underwent AS surgery as their early treatment, concomitant with PH-specific pharmacological treatment were reported. Patient’s hemodynamic and general condition improved with no further complications during the follow-up period. Atrial septostomy was usually conducted after all PH-specific pharmacological interventions failed. However, a study found that the survival benefit of AS was significantly increased if it was conducted before PH-specific pharmacotherapies. Most of the patients in this case received immediate hemodynamic and functional improvement. In this case series, it was reported that the AS procedure could lower the pulmonary arterial pressure and be safely conducted without further complications or death &gt;24 hr post-surgery. Considering the clinical benefit, safety procedure, and improved pulmonary arterial pressure, performing AS procedure concomitant with PH-specific pharmacotherapy as an early treatment for PH patients is recommended.

Pulmonary Hypertension Crisis in Patient with Tetralogy of Fallot and Mixed Total Anomalous Pulmonary Vein Connection after the Primary Correction: A Rare Case Report

Pulmonary Hypertension Crisis in Patient with Tetralogy of Fallot and Mixed Total Anomalous Pulmonary Vein Connection after the Primary Correction: A Rare Case Report

Pregnancy outcomes of women with Eisenmenger syndrome: A single-center study

BackgroundTo explore the outcomes of mothers with Eisenmenger syndrome (ES) and their offspring. MethodsPregnant women with ES admitted to the Beijing Anzhen Hospital between 2010 and 2019 were retrospectively analyzed and followed up. ResultsForty-two parturient women with ES were recruited, with an average age of 26.7 years (standard deviation [SD], ±4.0 years). The average gestational age was 33.7 weeks (SD, ±2.5 weeks). The average percutaneous oxygen saturation was 84.1 (±9.2), and 40 (95.2%) had caesarean delivery. The average pulmonary artery systolic pressure was 107.5 mmHg (SD, ±20.3 mmHg). Twelve (28.6%) women experienced pulmonary hypertensive crisis; 11 (26.2%) of these women died. Regarding the offspring, the average fetal weight was 1778.1 g (SD, ±555.3 g), six (14.3%) died, and congenital heart disease was diagnosed in three (7.1%). There were significant differences in age, gestational age, percutaneous oxygen saturation, Apgar score, and heart failure between the maternal death and non-death groups (P < 0.05). Death was mainly related to pulmonary hypertensive crisis and heart failure. ConclusionsWe recommend pregnancy termination if ES occurs during early pregnancy; however, patients should be informed of the risks if it occurs during late pregnancy. Multidisciplinary cooperation should be strengthened to improve the prognosis of the mothers and their offspring.

Causes of Death in Patients with Sickle Cell Disease from a Large Population-Based Cohort

Background Mortality rates and causes of death in children with sickle cell disease (SCD) have changed significantly over the past several decades. With ongoing improvements in standards of care, modern mortality rates and causes of death must be updated. Children's Healthcare of Atlanta (CHOA) houses one of the largest pediatric SCD programs in the country. This analysis reviews causes of death in children with SCD who were treated at CHOA between June 2010-June 2022. Methods We reviewed the CHOA's Sickle Cell Clinical Database (SCCD) for deceased patients. Demographics, SCD genotype, date and age at death, healthcare utilization, hydroxyurea (HU) use, chronic transfusion therapy (CTT), and bone marrow transplant (BMT) were obtained from the SCCD. Cause of death and history of significant comorbidities were abstracted from the medical record, death certificates and/or autopsy reports. Results A total of 4022 unique patients with SCD were seen at CHOA from June 2010- June 2022. Fifty-seven patients died during this period. All but 1 patient had been seen in the CHOA Sickle Cell Outpatient Clinic at least once. That patient received SCD care at a different institution, died from complications of a non-SCD related disease following transfer to CHOA, and was excluded from analysis. SCD-related causes accounted for 22 (39%) deaths. Acute SCD-related causes included bone marrow fat emboli, acute chest syndrome or pneumonia, cerebral hemorrhage in a person with history of stroke and moyamoya, and pulmonary hypertensive crisis. Of the infectious causes included Streptococcus pneumoniae, gram negative bacteria, unspecified streptococcal meningitis (by verbal report), and due to presumed septic shock complicated by severe anemia. Viral infections included respiratory syncytial virus, COVID-19 with severe anemia, and Parvovirus B-19 complicated by bone marrow fat emboli. Death due to complications of treatment included ceftriaxone-induced hemolysis and severe intrathoracic hemorrhage after apheresis catheter removal for treatment of ACS. BMT-associated complications included adenovirus infection after transplant with a matched unrelated donor and Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis after haploidentical transplant (see Figure 1). Non-SCD comorbidities or accidents accounted for 15 (27%) deaths. These included heart disease, advanced cancer or complications of cancer (including E. coli sepsis in a child with relapsed neuroblastoma), accidents, one due to motor vehicle accident and one drowning. Gastrointestinal causes included bleeding from undiagnosed inflammatory bowel disease and liver failure leading to coagulopathy and intracranial hemorrhage. There was a case of severe anemia related to systemic lupus erythematosus. The causes of death were unknown in the remaining 19 (34%) of individuals. Six (11%) were under 18 years of age. Five died at home or out of state and had no autopsy information, one had autopsy declined by the coroner. Of those who were 18 years of age or older, 7 (53%) were lost to follow-up, 3 (23%) had transitioned to adult care, and 3 (23%) were still being treated at CHOA. There were more deaths in the 0-2 year and 15-17 year groups, and the most among adults (n = 19, 34%). Death from congenital heart disease was more frequent in children 0-5 years old. Pneumococcal sepsis were in children 5-7 years old, after stopping penicillin and before the second Pneumovax vaccine. Other bacterial infections were evenly distributed by age. Non-infectious SCD-related deaths were more likely to occur in patients 12 years and older (n = 6, 86%) (Figure 2). Conclusions Continuing from a previous study by Lai et al. (2020) which reported the 10-year mortality rate at CHOA, this study described the causes of death of SCD patients during a 12-year period. Of direct SCD-related causes, bone marrow fat embolism was the most common and was also present in a patient who also had Parvovirus-B19 infection. Severe anemia was present in several patients at the time of death, associated with ceftriaxone-induced hemolysis, presumed septic shock, COVID-19, systemic lupus erythematosus, as well as G or intracranial hemorrhage. Patients beginning transition to adult care are at risk of becoming lost to follow-up and may experience difficulties transitioning to adult care. Future analyses will include review of regional hospital where available, and CDC's National Death Index. Figure 1View largeDownload PPTFigure 1View largeDownload PPT Close modal