Reversible severe pulmonary hypertension related to scurvy in children.

BACKGROUND: Severe 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 PRESENTATION: The 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. CONCLUSION: Pediatric 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.

Efficacy of interatrial shunt devices: an opening window to acute pulmonary hypertensive crisis and chronic pulmonary arterial hypertension.

The current study aimed to elucidate the efficacy of interatrial shunt device (IASD) for the treatment of acute pulmonary hypertensive crisis (PHC) and chronic pulmonary arterial hypertension (PAH). After establishing chronic PAH models using dehydrogenized monocrotaline (DHMCT), PAH dogs were implanted with IASDs (group A) or received no intervention (group B). One month later, DHMCT was injected again to establish an acute PHC. The prognosis, hemodynamics, ultrasound cardiography, electrocardiogram, and lung pathology of the dogs were observed. The baseline mean pulmonary arterial pressure increased from 12.70 ± 1.03 to 19.95 ± 1.75 mmHg and established a chronic PAH model 2 months after DHMCT injection (1.50 mg/kg). After an additional injection of DHMCT (1.50 mg/kg) in the chronic PAH model, acute PHC occurred. Mean PAP, sPAP, and pulmonary vascular resistance increased to 22.67 ± 1.80 mmHg, 35.70 ± 1.66 mmHg, and 12.50 ± 3.50 WOOD U, respectively. Cardiac output (CO) decreased to 1.31 ± 0.26 L/min, and the right-to-left shunt caused hypoxemia. The survival rates of the dogs with and those without IASD were 70.0% and 22.2% (P = 0.037), respectively. Six months after PHC, the CO between the dogs with and those without IASD were 1.44 ± 0.11 L/min and 1.18 ± 0.04 L/min (P = 0.028). The long-term survival rates were 50.0% and 22.2%, respectively (P = 0.21). IASD might be efficacious and beneficial for treating acute PHC and chronic PAH, as well as improving prognosis.

Perioperative management of patients with pulmonary hypertension undergoing non-cardiothoracic, non-obstetric surgery: a systematic review and expert consensus statement.

BACKGROUND: The risk of complications, including death, is substantially increased in patients with pulmonary hypertension (PH) undergoing anaesthesia for surgical procedures, especially in those with pulmonary arterial hypertension (PAH) and chronic thromboembolic PH (CTEPH). Sedation also poses a risk to patients with PH. Physiological changes including tachycardia, hypotension, fluid shifts, and an increase in pulmonary vascular resistance (PH crisis) can precipitate acute right ventricular decompensation and death. METHODS: A systematic literature review was performed of studies in patients with PH undergoing non-cardiac and non-obstetric surgery. The management of patients with PH requiring sedation for endoscopy was also reviewed. Using a framework of relevant clinical questions, we review the available evidence guiding operative risk, risk assessment, preoperative optimisation, and perioperative management, and identifying areas for future research. RESULTS: Reported 30 day mortality after non-cardiac and non-obstetric surgery ranges between 2% and 18% in patients with PH undergoing elective procedures, and increases to 15-50% for emergency surgery, with complications and death usually relating to acute right ventricular failure. Risk factors for mortality include procedure-specific and patient-related factors, especially markers of PH severity (e.g. pulmonary haemodynamics, poor exercise performance, and right ventricular dysfunction). Most studies highlight the importance of individualised preoperative risk assessment and optimisation and advanced perioperative planning. CONCLUSIONS: With an increasing number of patients requiring surgery in specialist and non-specialist PH centres, a systematic, evidence-based, multidisciplinary approach is required to minimise complications. Adequate risk stratification and a tailored-individualised perioperative plan is paramount.

Pulmonary hypertensive crisis: A potential reason for right ventricle and pacemaker lead failure.

Cardiac pacemakers have improved patient survival and quality of life, although malfunctions can be seen. We present the case of a girl with Seckel syndrome and congenital complete heart block. She had a single chamber permanent pacemaker in the right ventricle. When she referred us with a pulmonary hypertensive crisis (PHC), it was seen that the device was not pacing even in maximum threshold and pulse width values. After new epicardial lead implantation into the left ventricular apex, capture could be established again. For the cases presenting with capture failure, after eliminating lead-related problems and biochemical abnormalities, PHC should be kept in mind as a reason.

The CRASH report: emergency management dilemmas facing acute physicians in patients with pulmonary arterial hypertension.

Treatment of acute emergencies in patients with pulmonary arterial hypertension (PAH) can be challenging. In the UK and Ireland, management of adult patients with PAH is centred in eight nationally designated pulmonary hypertension (PH) centres. However, many patients live far from these centres and physicians in local hospitals are often required to manage PAH emergencies. A committee of physicians from nationally designated PH centres identified the 'most common' emergency clinical scenarios encountered in patients with PAH. Thereafter, a review of the literature was performed centred on these specified topics and a management approach was developed based on best available evidence and expert consensus. Management protocols were developed on the following PAH emergencies: chest pain (including myocardial ischaemia), right ventricular failure, arrhythmias, sepsis, haemoptysis ('CRASH'), as well as considerations relevant to surgery, anaesthesia and pregnancy. Emergencies are not uncommon in PAH. While expertise in PAH management is essential, all physicians involved in acute care should be aware of the principles of acute management of PAH emergencies. A multidisciplinary approach is necessary, with physicians from tertiary PH centres supporting care locally and planning safe transfer of patients to PH centres when appropriate.

Pulmonary hypertensive crisis and its efficient management. A Case report and literature review.

Perioperative anaesthetic management of patients with pulmonary hypertensive crisis is complex and challenging because of limited window period and treatment options. This case report describes the successful management of a patient with severe valvular heart disease superimposed by severe pulmonary hypertension, who developed pulmonary hypertensive crisis soon after induction of general anaesthesia for cardiac surgery. Pulmonary hypertensive crisis is not a usual phenomenon in these patients, but if it occurs it can be fatal. Anticipatory care of the patient to prevent crisis is the mainstay of treatment, otherwise it adds additional morbidity to such patients. Fortunately our patient survived the crisis without causing any additional morbidity during his hospital stay.

A case of malignant catatonia with idiopathic pulmonary arterial hypertension treated by electroconvulsive therapy.

BACKGROUND: Idiopathic pulmonary arterial hypertension (IPAH) is a progressive and fatal cardiovascular disease if left untreated. In patients with IPAH with psychiatric illness or other complications, careful attention is required when administering medical therapies that may affect their hemodynamics. Patients suffering from IPAH who undergo anesthesia and surgery have a high mortality and morbidity rate. We describe the treatment of intractable psychiatric symptoms with electroconvulsive therapy (ECT) in a patient with IPAH. CASE PRESENTATION: A 23-year-old woman with IPAH and type I diabetes mellitus (DM) presented with malignant catatonia. Her heart function was classified as New York Heart Association (NYHA) class III. She required a rapid cure and ECT due to various psychiatric symptoms resistant to conventional medications. Pulmonary hypertensive (PH) crisis is the most concerning complication that can be induced by the sympathetic stimulation of ECT. To avoid PH crisis, we administered oxygen using a laryngeal mask and administered remifentanil for anesthesia. We also prepared standby nitric oxide for possible PH crisis, although it was ultimately not needed. With 14 ECT sessions, her malignant catatonia was ameliorated without physical complications. CONCLUSION: ECT is an acceptable option for the treatment of medication-refractory psychiatric disturbances in patients with IPAH, provided careful management is assured to prevent or address complications.

Low cardiac output thyroid storm in a girl with Graves' disease.

A 15-year-old girl with Graves' disease presented with hypotension after methimazole and propranolol were re-started for hyperthyroidism. She was found to have pulmonary artery hypertension resulting in obstructive shock. Thyroid storm was diagnosed according to Burch and Wartofsky score. She was promptly treated with anti-thyroid drugs, inorganic iodide, corticosteroid, and respiratory support. Pulmonary hypertension was treated with inhaled nitric oxide until the clinical status improved. Propranolol was withdrawn due to poor cardiac function. We herein present a unique case of a difficult-to-treat Graves' disease presenting with severe pulmonary hypertension resulting in low cardiac output thyroid storm.

Effect of phosphodiesterase type 5 inhibitors on surgical outcome of ventricular septal defect and pulmonary hypertension patients.

BACKGROUND: Children with ventricular septal defect (VSD) and large systemic-to-pulmonary shunts eventually develop pulmonary hypertension (PH). The perioperative management of patients with VSD and PH is quite troublesome and still debatable, especially in developing countries where the different management options and standardization of treatment is not available. Oral phosphodiesterase type 5 (PDE-5) inhibitors are good treatment options being widely available, cheap, easy administration and do not require extensive monitoring. The aim of our study was to evaluate the effect of the PDE-5 inhibitors when given orally, early preoperative and continued for 3 months postoperative on controlling postoperative PH with its effect on right ventricle (RV) functions. Fifty-one patients were randomly assigned to either sildenafil or tadalafil, 1 week before and continued for 3 months after corrective surgery. The control group received a placebo. RESULTS: There was no significant difference in the improvement in the right ventricle systolic pressure (RVSP) between both groups, early in the postoperative period (P = 0.255) and in follow-up (P = 0.259). There was also no significant difference in the changes in mean pulmonary artery pressure (mPAP), postoperatively and on follow-up (P = 0.788 and 0.059, respectively). There was a drop in RV functions in both groups postoperatively which improved on follow-up; however, it was not significant between both groups. The length of intensive care unit (ICU) stay was similar between both groups (P = 0.143). CONCLUSION: Perioperative administration of PDE-5 inhibitors does not have an impact on the clinical course as regards improvement in pulmonary artery (PA) pressure, ventricular functions and ICU stay.

Postoperative pulmonary hypertensive crisis caused by inverted left atrial appendage after cardiopulmonary bypass surgery for congenital heart disease in a neonate.

Postoperative pulmonary hypertensive crisis (PHC) caused by an inverted left atrial appendage (ILAA) is a rare complication following cardiac surgery. We present a case of 23 day-old male infant who developed postoperative PHC attacks after undergoing cardiopulmonary bypass (CPB) surgery for repair of the coactation of aorta. A hyperechogenic left atrial mass was detected via bedside transthoracic echocardiography (TTE), which was identified as an ILAA and corrected following repeat surgery. In this case, both the negative pressure in vent catheter and the long left atrial appendage (LAA) with a narrow base led to an irreversible ILAA. As in this neonate, ILAA had significant influence on the left atrial volume and caused PHC since the ILAA was located on the mitral valve orifice and interfered with the blood flow through the valve. Therefore, we recommend that the vent catheter should be turned off before removing to avoid this potential complication. Additionally, LAA should be carefully inspected after CPB surgery, and intra-operative and post-operative transoesophageal echocardiography (TEE) should be performed to detect ILAA intraoperatively so as to avoid the reoperation. When an ILAA is diagnosed postoperatively, whether conservative treatment or surgery will depend on the balance of benefit and risk for a particular patient.

A 1-month-old infant with pulmonary hypertensive crisis after cardiac surgery was successfully rescued with connection of left atrial and right ventricular pressure measurement tubes: a case report.

Background: Pulmonary hypertensive crisis is a complication with extremely high mortality after surgery of congenital heart disease. However, there are still no treatment guidelines or expert consensus on the standard treatment of pulmonary hypertensive crisis, and the effect of conventional treatment is still unsatisfactory. We present a case of a patient who developed pulmonary hypertensive crisis after cardiac surgery, and was successfully rescued with a pioneering method, which has never been reported so far. Case summary: An infant with congenital heart disease had undergone cardiac surgery successfully. Due to obvious myocardial oedema, sternal closure was delayed. The left atrial and right ventricular pressure monitoring tubes, both of which were connected through a triplet, were inserted into right pulmonary vein and pulmonary artery, respectively, and the triplet was in closed condition. On the night of the surgery, pulmonary hypertensive crisis occurred. Emergency bedside thoracotomy was given, and the triplet was turned on urgently to make the left atrial and right ventricular pressure monitoring tubes connected. Meantime, conventional treatment was performed. Eventually, the pulmonary hypertensive crisis was quickly relieved, and the infant was discharged 9 days later. Discussion: The left atrial and right ventricular pressure monitoring tubes are placed intraoperatively in patients who both need delayed sternal closure and have high risk factors for pulmonary hypertensive crisis, by which could not only monitor the pressure of left atrium and right ventricle in real time but also effectively relieve the right ventricular pressure instantaneously when pulmonary hypertensive crisis occurs, as well as remedy ischaemia of systemic and coronary circulation.

Factors Associated with Early Postoperative Results of Total Anomalous Pulmonary Venous Connection Repair: Findings from Retrospective Single-Institution Data in Vietnam.

INTRODUCTION: There are scanty reports of the risk factors for pulmonary hypertensive crisis and low cardiac output syndrome after the operative repair of total anomalous pulmonary venous connection (TAPVC). We aim to evaluate early surgical outcomes of TAPVC and risk factors for pulmonary hypertensive crisis and low cardiac output syndrome. METHODS: We conducted a retrospective medical record review for all patients undergoing operative repair of TAPVC within 5 years. Outcome variables included pulmonary hypertensive crisis, low cardiac output syndrome and early mortality. RESULTS: Of 58 patients, we documented 77.59% supracardiac, 20.69% cardiac and 1.72% mixed site of connection. About 86.21% patients underwent elective surgery, and 13.79% patients required emergency surgery. Incidence rates were 27.59% for pulmonary hypertensive crisis and 6.90% for low cardiac output syndrome. Body weight below 6 kg, pneumonia, tachycardia, hepatomegaly, preoperative pulmonary congestion on chest x-ray, preoperative elevated mean pulmonary artery pressure, preoperative pulmonary venous obstruction, emergency surgery and prolonged aortic cross-clamping time were significant risk factors for postoperative pulmonary hypertensive crisis. Significant risk factors for postoperative low cardiac output syndrome included pneumonia, prolonged duration of preoperative mechanical ventilation and prolonged aortic cross-clamping time. CONCLUSION: The early outcome of surgical repair of TAPVC was acceptable, with 96.55% survival rate. This current analysis suggests that a thorough evaluation of all preoperative and operative characteristics is imperative to achieve best medical and surgical outcomes.

Perioperative management of a patient with double orifice mitral valve with supramitral ring with subaortic membrane with ventricular septal defect and severe pulmonary hypertension: Report of a rare case.

Double-orifice mitral valve (DOMV) is an unusual congenital anomaly characterized by a mitral valve with a single fibrous annulus with two orifices or rarely two orifices with two separate mitral annuli opening into the left ventricle. We present a first report of a patient with a DOMV with supramitral ring (SMR), subaortic membrane (SAM), a large ventricular septal defect (VSD) with more than 50% aortic override, and severe pulmonary arterial hypertrophy (PAH). This patient underwent excision of the SAM, and SMR, with closure of the VSD together under cardiopulmonary bypass (CPB). However postoperatively, the patient developed an irreversible fatal pulmonary hypertensive crisis (PHC), immediately after transferring the patient to the cardiac intensive care unit from the operating room (OR). The PHC was refractory to intravenous and inhaled milrinone and nitroglycerine and intravenous adrenaline, dobutamine, norepinephrine, vasopressin, patent foramen oval (PFO), and CPB support. The management of DOMV and perioperative pulmonary hypertension is discussed.

Advanced Pulmonary Hypertension Due to Congenital Double-shunt Successfully Treated with Surgical Repair and Up-front Combination Therapy.

This case report concerns a 22-year-old woman with large patent ductus arteriosus and atrial septal defect. She was referred to our hospital because of exertional dyspnea and was revealed to have advanced pulmonary arterial hypertension (PAH) with a mean pulmonary arterial pressure (PAP) of 79 mmHg. Although both shunts had bidirectional flow, based on the results of acute pulmonary vasoreactive testing, one-stage surgical closure was performed followed by up-front combination therapy for post-operative pulmonary hypertensive crisis and residual PAH. At 14 months after the surgery, her symptoms were markedly improved, and her mean PAP had dramatically decreased to 13 mmHg.

Pediatric Perioperative Pulmonary Arterial Hypertension: A Case-Based Primer.

The perioperative period is an extremely tenuous time for the pediatric patient with pulmonary arterial hypertension. This article will discuss a multidisciplinary approach to preoperative planning, the importance of early identification of pulmonary hypertensive crises, and practical strategies for postoperative management for this unique group of children.

Iloprost for children with pulmonary hypertension after surgery to correct congenital heart disease.

Congenital heart disease (CHD) can cause pulmonary hypertension (PH) in children, and surgery to correct CHD may be complicated by postoperative pulmonary hypertensive crises (PHC). Clinical data regarding the use of inhaled iloprost to treat children with PH are scarce. Our aim was to determine the efficacy and safety of iloprost in children with PH following surgery to correct CHD. This was a randomized, placebo-controlled study of 22 children (median age 7 months) undergoing surgery to achieve biventricular repair. The combined clinical endpoint was a decrease of more than 20% in the ratio of systolic pulmonary arterial pressure to systolic arterial pressure or pulmonary resistance to systemic resistance, with no PHC or death. Patients were randomized to receive low-dose iloprost (30 ng/kg/min), high-dose iloprost (50 ng/kg/min), or placebo, for 10 min every 2 hr in the first 48 hr after surgery. PHC were experienced by two patients who received placebo and one patient treated with high-dose iloprost. The combined clinical endpoint was reached by six patients administered low-dose iloprost (P = 0.005) and four administered high-dose iloprost (P = 0.077), compared with none in the placebo group. Patients treated with iloprost showed a significant reduction from baseline in mean pulmonary vascular resistance index (-2.2 Wood units, P < 0.05), whereas patients who received placebo showed no significant change. This study supports the use of iloprost to treat children with PH following surgery to correct CHD.

Perioperative pharmacological management of pulmonary hypertensive crisis during congenital heart surgery.

Pulmonary hypertensive crisis is an important cause of morbidity and mortality in patients with pulmonary arterial hypertension secondary to congenital heart disease (PAH-CHD) who require cardiac surgery. At present, prevention and management of perioperative pulmonary hypertensive crisis is aimed at optimizing cardiopulmonary interactions by targeting prostacyclin, endothelin, and nitric oxide signaling pathways within the pulmonary circulation with various pharmacological agents. This review is aimed at familiarizing the practitioner with the current pharmacological treatment for dealing with perioperative pulmonary hypertensive crisis in PAH-CHD patients. Given the life-threatening complications associated with pulmonary hypertensive crisis, proper perioperative planning can help anticipate cardiopulmonary complications and optimize surgical outcomes in this patient population.

Prevention and treatment of perioperative pulmonary hypertensive crisis in patients with serious pulmonary arterial hypertension relevant with ventricular septal defect / 中国实用护理杂志

Objective To discuss key points of prevention and treatment of perioperative pulmonary hypertensive crisis in patients with serious pulmonary arterial hypertension associated with ventricular septal defect. Methods Retrospectively analyzed the nursing experience of perioperative pulmonary hypertensive crisis on 31 patients with serious pulmonary arterial hypertension associated with ventricular septal defect from March to December during 2016.Among these patients,7 patients occurred pulmonary hypertensive crisis.The prevention contained avoiding oxygen lack,keeping pH alkaloid in the body, application of pulmonary vasodilator, deep sedation. Results A total of 30 cases survived the perioperative period, and were discharged from the hospital, one died. Conclusions The patients with serious pulmonary arterial hypertension had more risks during the perioperative period,the main cause of death was pulmonary hypertensive crisis during this time.So prevention of pulmonary hypertension crisis is the key point of postoperative nursing.

Treatment of Pulmonary Hypertensive Crisis Using ECMO: A Case Report / 대한흉부외과학회지

Extracorporeal membrane oxygenation(ECMO) provides stable oxygenation to prevent elevation of pulmonary vascular resistance and bypasses a significant part of cardiac output to the pulmonary vascular bed to reduce pulmonary perfusion pressure. In addition, ECMO prevents right heart failure and low cardiac output by means of ventricular assist and reduction in volume load to right ventricle. As a result, ECMO can be used for the treatment of pulmonary hypertensive crisis after surgery for congenital heart disease, especially when it is refractory to conventional measures. We report a case of postoperative pulmonary hypertensive crisis, developed in a 37-year-old male with patent ductus arteriosus with secondary pulmonary hypertension, which was successfully managed including ECMO.