Among congenital cyanotic heart diseases (CHDs), situs inversus totalis with transposition of great vessels with a large ventricular septal defect (VSD) has a very low incidence of around 1 in 10,000. Hereby, we present a 16-year-old man with the aforementioned cardiac anomaly with cardiac arrhythmias, septic shock, and a history of road traffic accident-causing osteomyelitis of the left thigh requiring incision and drainage. The patient was admitted to the intensive care unit with a high-grade fever, narrow pulse pressure, and atrial fibrillation. The patient was operated on under general anesthesia with endotracheal intubation after optimization. Invasive monitoring, antiarrhythmics, and vasopressors were required intraoperatively, and surgery progressed uneventfully. Furthermore, the patient had undergone a series of debridements after 8 days, which were performed under regional anesthesia uneventfully. This case report represents a plan of action for perioperative anesthetic management and anticipates the difficulties for CHD patients in the course of surgery and subsequential prudence.

BACKGROUND: Myopathy, lactic acidosis and inherited sideroblastic anemia (MLASA) are a group of rare intriguing disorders with wider pathophysiological implications. One of the causes of MLASA is the mutation in PUS1 gene that encodes for pseudouridine synthase. This PUS1 mutation results in MLASA in which anemia and myopathy predominate. Severe pulmonary arterial hypertension has not been previously reported in patients with PUS1 gene mutation.
METHODS: A 17 year old girl with congenital sideroblastic anemia presented with worsening of breathlessness. Severe pulmonary artery hypertension was documented on investigations. A homozygous variant in exon 3 of gene PUS1,( chromosome 12:g.131932301 C > T c.430 C > T) was found on sanger sequencing.
CONCLUSIONS: We document severe pulmonary arterial hypertension in a patient of congenital sideroblastic anemia from PUS1 gene. We hypothesis that cross talk with TGFb pathways might occur in PUS1 mutation, and that might cause severe PAH. This observation might have therapeutic implications.

Atrial septal defects (ASDs), comprising a significant portion of congenital cardiac anomalies, encompass a rarer and more diagnostically challenging subset known as sinus venosus ASDs (SVASDs). ASDs are more prevalent in females, and the prognosis for patients under 40 years of age is generally favorable with advancements in surgical and transcatheter interventions. However, undiagnosed ASDs in adults above 40 years old, especially females, often lead to severe complications, including pulmonary hypertension, atrial fibrillation, Eisenmenger syndrome, and a mortality rate exceeding 50%. Our detailed case study focuses on an obese 42-year-old Hispanic migrant female with chronic respiratory failure misattributed to pulmonary hypertension, resulting in the progression of complications from undiagnosed SVASD. Further investigation using contrast-enhanced transesophageal echocardiography (TEE) elucidated the correct diagnosis four years after her initial presentation. This report explores the potential factors contributing to the patient\'s delayed diagnosis and development of advanced cardiac complications of pulmonary hypertension leading to Eisenmenger syndrome that precluded her from procedural intervention. Furthermore, this report pioneers the first thorough review of case reports in adults newly diagnosed with SVASD, revealing sex-based differences in complications.

Sickle cell disease (SCD) is associated with substantial morbidity and early mortality in afflicted adults. Cardiopulmonary complications that occur at increased frequency in SCD such as pulmonary embolism, pulmonary arterial hypertension, and acute chest syndrome can acutely worsen right ventricular function and lead to cardiogenic shock. Mechanical circulatory support including venoarterial extracorporeal membrane oxygenation (VA ECMO) is being increasingly utilized to treat hemodynamic collapse in various patient populations. However, a paucity of literature exists to guide the use of mechanical circulatory support in adults with SCD where disease-related sequela and unique hematologic aspects of this disorder may complicate extracorporeal therapy and must be understood. Here, we review the literature and describe three cases of adult patients with SCD who developed cardiogenic shock from acute decompensated right heart failure and were treated clinically with VA ECMO. Using an in vitro ECMO system, we investigate a potential increased risk of systemic fat emboli in patients with SCD who may be experiencing vaso-occlusive events with bone marrow involvement given the high-volume shunting of blood from venous to arterial systems with VA ECMO. The purpose of this study is to describe available extracorporeal life support experiences, review potential complications, and discuss the special considerations needed to further our understanding of the utility of VA ECMO in those with SCD.

BACKGROUND: Priapism is defined as erection that lasts for more than 4 h without sexual stimulation. There are various causes of priapism, but there are no reports of sildenafil-induced priapism in dogs. In human medicine, there were no pre-marketing reports of priapism caused by sildenafil, but post-marketing surveillance has shown that it is rare. In cases of pulmonary hypertension in dogs, sildenafil is the first-line drug of choice for symptomatic relief.
METHODS: An 11-year-old neutered male Maltese dog that presented with tachypnea and cough was diagnosed with myxomatous mitral valve disease, American College of Veterinary Internal Medicine (ACVIM) stage C, and was treated medically. Eighteen months after the diagnosis, severe pulmonary hypertension occurred due to left heart disease. At 20 months postdiagnosis, pleural effusion occurred, and sildenafil (2 mg/kg twice daily) was added to the existing treatment. Two weeks later, the dyspnea recurred, confirming pleural fluid recurrence, and sildenafil was increased to 2 mg/kg thrice daily. One day later, the patient developed persistent erections and penile pain. Penile amputation and urethrostomy were recommended but were refused; therefore, analgesia and palliative care were provided. The patient died of acute dyspnea 22 months after the first presentation, with no specific priapism recurrence at the time of death.
CONCLUSIONS: To the best of our knowledge, this is the first report of sildenafil-induced priapism in a dog with pulmonary hypertension.

Severe pulmonary hypertension (PH) during pregnancy poses considerable challenges due to the physiological changes and increased cardiovascular demands. Close multidisciplinary management is essential throughout the peripartum period. The critical steps taken to provide anesthesia safely and successfully for a planned cesarian section are outlined, with special care for communication between the cardiothoracic surgery and obstetric team. A 31-year-old G3P1112 (three pregnancies, one term delivery, one pre-term delivery, one abortion, with two living children) patient with a history of systemic lupus erythematosus complicated by Group 1 PH presented to the operating room for a planned 34-week cesarean section. Pulmonary artery systolic pressure (PASP) was noted to be 68 mmHg at this time. Intravenous (IV) treprostinil at 8 ng/kg/min through a tunneled right subclavian line was initiated in her third trimester, and a day before her cesarean section, she was admitted for a lumbar epidural catheter placement. In the operating room, IV treprostinil was continued and a high-flow nasal cannula with inhaled nitric oxide at 20 ppm was initiated. A right internal jugular vein pulmonary artery catheter was placed for close monitoring of her pulmonary artery pressures, with a PASP reading of 64 mmHg at the start of the case. Femoral arterial and venous access was placed by the cardiothoracic surgery team for cardiopulmonary bypass standby. Intra-operative surgical analgesia was achieved by epidural lidocaine. A cesarean section was performed and was uncomplicated despite her post-delivery autotransfusion, where her PASP went as high as 89 mmHg. Uterine atony was managed with an oxytocin infusion. Epidural morphine was administered through the epidural catheter for post-operative analgesia. In the post-operative recovery room, her PASP was back down to baseline at 62 mmHg. The patient proceeded to have an uneventful postpartum hospital stay and was discharged home without any complications. While severe PH poses a challenge in the care of a parturient patient, safe and successful management may be achieved as outlined in this case report.

OBJECTIVE: To retrospectively evaluate the incidence rate (IR) of elevated echocardiographic estimated systolic pulmonary artery pressure (sPAP), suspected for pulmonary hypertension (PH), in systemic sclerosis (SSc) patients after the introduction of a combination therapy with bosentan and sildenafil for treatment or prevention of digital ulcers.
METHODS: Patients attending the Scleroderma Unit of the Universital Hospital of Careggi from July 2010 to July 2023 were enrolled. Patients older than 18 years old with a history of digital ulcers, treated with bosentan and sildenafil in combination for at least 12 months, were included. Patients with a diagnosis of PH preceding the introduction of the therapy were excluded. Demographical data, disease duration, laboratoristic, and instrumental data (pulmonary function tests, echocardiographic estimation of sPAP, and ultrasonographic value of renal resistive index) were collected. The IR of echocardiographic signs suspected of pulmonary hypertension and their 95% confidence interval were calculated in events/1000 patients-years.
RESULTS: Thirty-five patients were enrolled; the mean disease duration was 12.82 years (SD 5.92). The mean duration of the combination treatment was 81.03 (SD 43.1.3) months, and the total at-risk time was 2674 months. Two patients (5.7%) presented echocardiographic signs of PH (sPAP 50 mmHg and 40 mmHg); the IR was calculated to be 9/1000 patients-years (95% CI 7.95-10.12). In one of the two patients, right heart catheterism (RHC) excluded PAH, while the other patient refused to undergo RHC, and PAH could not be confirmed/excluded. The stability of PFTs and echocardiographic sPAP was observed during the observation time.
CONCLUSIONS: The results of this retrospective study suggest that combination therapy with endothelin receptor antagonists and phosphodiesterase-5 (PDE5) inhibitors could help in preventing PAH in SSc; prospective case-control studies on a larger population are needed to improve knowledge in this field.

UNASSIGNED: Hereditary hemorrhagic telangiectasia (HHT) is an uncommon autosomal dominant condition. The combination of pregnancy and HHT can exacerbate pulmonary hypertension (PH) and, in severe cases, lead to fatality.
UNASSIGNED: The case we presented is a 28-year-old multiparous woman. She developed chest tightness and dyspnea in the second trimester of pregnancy, which was not taken seriously at that time, and the symptoms worsened postpartum. Echocardiography showed elevated pulmonary artery pressure (PAP) and the computerized tomographic pulmonary angiogram revealed a significant pulmonary arteriovenous malformation. The patient\'s condition continued to deteriorate despite treatment to reduce pulmonary hypertension. We reviewed and updated the history of omission, recurrent epistaxis during pregnancy, and similar symptoms running in her family. Combined with the whole exon genetic testing report revealing the ACVRL1 gene mutation at chr12:52308295, the diagnosis of HHT was established. Four months later, a transcatheter closure of the pulmonary arteriovenous fistula was performed, with satisfying outcomes presenting a decrease of more than 15 mmHg in the pulmonary artery pressure. As of right now, the patient\'s status is stable during the outpatient follow-up.
UNASSIGNED: HHT is a rare condition that typically occurs alongside abnormal communication between pulmonary veins and arteries, leading to a high-flow state in the pulmonary circulation. A pulmonary hypertension crisis can also be triggered by the patient\'s pregnancy, which further increases blood volume. By reducinhttps://www.ecdc.europa.eu/sites/default/files/documents/Methods-for-the%20detection-and-characterisation-of-SARS-CoV-2-variants-first-update-WHO-20-Dec-2021.pdfg pulmonary vascular flow, catheter closure of the pulmonary arteriovenous fistula decreases pulmonary arterial pressure.

BACKGROUND: Sarcoidosis-associated pulmonary hypertension (SAPH) is listed in Group 5 of the clinical classification of pulmonary hypertension, due to its complex and multifactorial pathophysiology. The most common cause of SAPH development is advanced lung fibrosis with the associated destruction of the vascular bed, and/or alveolar hypoxia. However, a substantial proportion of SAPH patients (up to 30%) do not have significant fibrosis on chest imaging. In such cases, the development of pulmonary hypertension may be due to the lesions directly affecting the pulmonary vasculature, such as granulomatous angiitis, pulmonary veno-occlusive disease, chronic thromboembolism or external compression of vessels by enlarged lymph nodes. Based on the case of a 69-year-old female who developed SAPH due to pulmonary arteries stenosis, diagnostic difficulties and therapeutic management are discussed.
METHODS: The patient, non-smoking female, diagnosed with stage II sarcoidosis twelve years earlier, presented with progressive dyspnoea on exertion, dry cough, minor haemoptysis and increasing oedema of the lower limbs. Computed tomography pulmonary angiography (CTPA) showed complete occlusion of the right upper lobe artery and narrowing of the left lower lobe artery, with post-stenotic dilatation of the arteries of the basal segments. The vascular pathology was caused by adjacent, enlarged lymph nodes with calcifications and fibrotic tissue surrounding the vessels. Pulmonary artery thrombi were not found. The patient was treated with systemic corticosteroid therapy and subsequently with balloon pulmonary angioplasty. Partial improvement in clinical status and hemodynamic parameters has been achieved.
CONCLUSIONS: An appropriate screening strategy is required for early detection of pulmonary hypertension in sarcoidosis patients. Once SAPH diagnosis is confirmed, it is crucial to determine the appropriate phenotype of pulmonary hypertension and provide the most effective treatment plan. Although determining SAPH phenotype is challenging, one should remember about the possibility of pulmonary arteries occlusion.

Pulmonary hypertension (PH) is a known and life limiting complication of preterm born young adults with bronchopulmonary dysplasia (BPD), ultimately leading to progressive right ventricular (RV) failure. Prognosis remains poor, especially in patients unresponsive to modern vasoactive pharmacotherapy. Therefore, lung transplantation presents the treatment of choice to avert cardiac failure. With limited donor organ availability and long waiting times, the implantation of a paracorporeal lung assist device (PLAD) is a way to bridge the patient as an alternative to veno-arterial ECMO. Herein, we present the case of a prematurely born 23-year-old female, who developed severe PH due to BPD and consequently experienced therapy refractory RV failure. Urgent PLAD implantation was performed and the patient successfully underwent double-lung transplantation after 215 days of PLAD support. No major PLAD-associated complications occurred and full recovery of RV function could be observed after double-lung transplantation.