Postoperative Pulmonary Hypertensive Crisis Research Articles

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.

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.

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.

Outcomes and occurrence of post-operative pulmonary hypertension crisis after late referral truncus arteriosus repair.

BackgroundTruncus arteriosus (TA) is a rare congenital heart disease with a high rate of early mortality. The occurrence of post-operative pulmonary hypertension crisis (PHC), known to be a common and life-threatening complication, increases due to the irreversible development of pulmonary vascular resistance with age. We sought to figure out the risk factors for PHC and describe the surgical outcomes of TA patients with late referral (repair <1 month excluded).Materials and methodsWe retrospectively reviewed patients after TA repair between 2009 and 2021 at Shanghai Children’s Medical Center. The occurrence of PHC was defined according to post-operative Pp/Ps ≥ 1 and clinical manifestations. Risk factors for PHC and mortality were conducted by multivariable analysis.ResultsA total of 98 patients were treated, including 55 males and 43 females. The median age at repair was 121 (69, 245) days. Post-operative PHC occurred in 22 (22.4%) patients with a median age of 186 (122, 293) days. By multivariable analysis, patients with the sum of Z-score of pre-operative bilateral pulmonary artery (PA) diameters (OR: 1.6, 95% CI: 1.2–2.3, P = 0.01) was more likely to experience PHC. Longer CPB duration contributed to early death (OR: 1.0, 95% CI: 1.0–1.0, P = 0.01). Total survival at 10 years was 81.4%. In 4.5 (2.9, 7.5) years of follow-up, twenty-six patients received 30 reinterventions. Valved reconstruction of RVOT most predicted reinterventions (OR: 4.2, 95% CI: 1.4–13.0, P = 0.01).ConclusionSurgical repair of TA patients with late referral has resulted in comparatively favorable early and mid-term outcomes. PHC occurred more commonly in patients with overextended bilateral PA pre-operatively. Meanwhile, valved reconstruction of RVOT would more likely lead to early reintervention.

Repair of common arterial trunk: palliation and delayed correction as a viable alternative strategy in selected patients.

OBJECTIVESPrimary repair of common arterial trunk (CAT) is burdened by high mortality rates, especially in the presence of multiple risk factors. Timing, possible palliative methods, optimal management of associated cardiac lesions and handling of a poor preoperative state are still under discussion.METHODSWe retrospectively analysed all patients who underwent surgery for CAT in our institution between 2008 and November 2020. We included 22 patients, 11 of whom received primary correction (PC) and 11 of whom underwent initial palliation by partial repair, leaving the ventricular septal defect open and connecting the right ventricle to the pulmonary arteries with a small valveless right ventricle-to-pulmonary artery conduit. A delayed correction (DC) was performed after 11.5 ± 3.6 months.RESULTSThe overall operative mortality was 4.5%; 1 patient (affected by severe truncal valve stenosis and presenting in a poor state preoperatively) in the DC group died after palliation. The incidence of postoperative pulmonary hypertensive crisis was significantly higher in the PC group (P = 0.027). No patient from either group required postoperative extracorporeal support. Survival rates after 6 years differed slightly (PC group, 90%; DC group, 70%; log-rank = 0.270).CONCLUSIONSPC of CAT remains an optimal surgical approach for patients with an expected low mortality. However, our data support palliation and DC as a suitable alternative strategy, especially in the presence of significant risk factors like interrupted aortic arch, poor preoperative condition or complex surgical anatomy.

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

IntroductionThere 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.MethodsWe 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.ResultsOf 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.ConclusionThe 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.

Transcatheter closure of patent vertical vein after repair of total anomalous pulmonary venous connection: a case series.

Total anomalous pulmonary venous connection is a rare cyanotic CHD that requires surgical repair. An unligated vertical vein after total anomalous pulmonary venous connection surgery may help to decrease the episodes of post-operative pulmonary hypertensive crises, low cardiac output syndrome, and mortality. The aim was to assess long-term outcome and our post-operative transcatheter vertical vein closure experiences in five patients with repaired total anomalous pulmonary venous connection patients. A retrospective study was conducted in five cases with an unligated vertical vein following repair of supra-cardiac total anomalous pulmonary venous connection at our hospital from 2011 through 2018. Patients characteristics, cardiac catheterisation findings, surgical, and transcatheter procedural details were retrospectively analysed. Transcatheter closure of the unligated vertical vein was technically successful in all the patients. Procedure-related complications were not observed in any of the patients. No long-term complication was found. We suggest that transcatheter closure of the patent vertical vein is an effective and well-tolerated alternative to the surgical approach.

Long-Term Outcomes of Total Anomalous Pulmonary Venous Drainage Repair in Neonates and Infants

Outcomes of operations for total anomalous pulmonary venous drainage (TAPVD) have improved. However, operations in the neonatal period and the development of postoperative pulmonary venous obstruction are associated with a high mortality rate. A retrospective review was conducted for all neonates and infants (n= 214) who underwent operations for isolated TAPVD (1973 to 2014). Median age was 18 days (1 day to 1 year). There were 17 (7.9%) early deaths. Risk factors for early death were prolonged cardiopulmonary bypass time (p= 0.005) and neonatal age at the operation (p= 0.048). Early mortality was 2.5% for infants (n= 81) and 11% for neonates (n= 133; p= 0.021) during the entire study period. Hospital deaths for neonates remained unchanged during the four eras of 1973 to 1988, 1989 to 1998, 1999 to 2008, and 2009 to 2014. Survival at 10 and 20 years was 88% ± 2.2% (95% confidence interval, 82% to 91%). Reoperation for postoperative pulmonary venous obstruction was required in 22 patients (10%). Risk factors for reoperation were prolonged cardiopulmonary bypass time (p= 0.015), lower operative weight (p= 0.003), and an episode of postoperative pulmonary hypertensive crisis (p= 0.005). Freedom from reoperation at 20 years was 86% ± 3.2% (95% confidence interval, 78% to 91%). All survivors were asymptomatic at a mean of 13 ± 9 years (range, 1 month to 42 years) after the operation. Although isolated TAPVD repair in infants can be performed without death, the operation is associated with a high mortality rate in neonates that remained unchanged during the long study period. Survival beyond 1 year after the operation is associated with excellent long-term outcomes.

The role of preoperative sildenafil therapy in controlling of postoperative pulmonary hypertension in children with ventricular septal defects.

Introduction: Most of the ventricular septal defects (VSD) are complicated with pulmonary arterial hypertension (PAH) which is the major cause of pulmonary hypertensive crisis and right ventricular failure. Methods: We reviewed clinical outcomes of 63 infants who underwent cardiac surgery and were divided into three groups. Control group (n=20) did not received sildenafil while group A (n = 22) received drug (0.3 mg/kg) before and after surgery. Group B (n=21) received drug at the initiation of surgery. Demographic data, preoperative and postoperative variables were compared among the patients. Results: Patients in the group A had lower preoperative pulmonary arterial pressure (PAP) compared to other groups (P < 0.001). Also, patients in control group had longer cardiopulmonary bypass time (P < 0.05). Postoperative PAP in patients of group A and B decreased significantly compared to control group (P < 0.001). Also, pre- and postoperative PVR (pulmonary vascular resistance) showed a significant decrease in group A compared with control and group B (P < 0.001). The intubation time in patients of the control group was significantly more prolonged compared with patients of group A and B (P < 0.001). Moreover, the length of ICU stay was significantly longer in patients of control group compared with group A and B (P < 0.001). Conclusion: Preoperative sildenafil therapy seems to be effective and safe to prevent postoperative PAH and pulmonary hypertensive crisis in children with ventricular septal defects and has a positive impact on postoperative care.

Left-to-Right Shunts

Contrary to the opinion of French pediatrician Henri-Louis1 that isolated ventricular septal defects (VSDs) are universally well tolerated, the subsequent discoveries of Eisenmenger,2 Abbott,3 Wood,4 and others have taught us that the prognosis of left-to-right shunt lesions is not always benign. After more than a century of research, as pediatric cardiologists and congenital heart surgeons, we claim that we know what happens in the lungs and the hearts of these patients and when these changes occur. The repair of VSDs has become routine since Lillehei’s, Warden’s, and Kirklin’s5,6 pioneering work in the 1950s, and we feel that we know in whom, at what age, and for which signs and symptoms to intervene. In reality, significant heterogeneity prevails between different centers and among individual physicians with regard to whether and when to close an isolated moderately restrictive VSD or patent arterial duct. Conceptually, we agree to occlude left-to-right shunts for one or both of the 2 reasons: to treat heart failure and to prevent irreversible pulmonary vascular damage, both of which have been identified as important risk factors in congenital heart disease. The study by Pereda et al7 from Madrid and Barcelona, published in this issue of Circulation: Cardiovascular Imaging , leads us to consider the fate of the myocardium as well. When counseling parents, we point out the low operative mortality and excellent long-term prognosis after successful VSD or patent arterial duct closure. Indeed, postoperative pulmonary hypertensive crises and more than transient and mild ventricular dysfunction are rare occurrences after shunt repair in infancy in the current era. Nonetheless, most of us vividly remember the stormy early postoperative course in the occasional …

A Prospective Randomised Study of Postoperative Prophylactic Application of Sildenafil After Pediatric Cardiac Surgery

Postoperative aggravation of pulmonary hypertension after pediatric cardiac surgery due to temporary endothelial dysfunction may lead to sudden pulmonary arterial pressure rise with compromise of right ventricular function. Though treatment with inhaled nitric oxid (NO) has been successful, rebound pulmonary hypertension has been observed after NO withdrawal due to supression of endogenous NO sythase. Sildenafil has been shown to prevent this reaction and is widely used after pediatric cardiac surgery. The aim of our study was to evaluate the effectively of postoperative administration of oral sildenafil (1 mg/kg/day) in preventing postoperative pulmonary hypertensive crises. 21 patients after repair of complete atrioventricular canal (n=10) or ventricular septal defect (n=11) were randomly assigned to the study group or control group. The study group received sildenafil after surgery via nasogastric tube every eight hours for 48 hours, the control group received placebo. The primary outcome parameter was the number of pulmonary hypertensive events (spontaneous pulmonary arterial pressure rise to or above systemic arterial pressure). Secondary outcome parameters were: need for inotropic drugs, fluid balance, urinary output, days on the ventilator or in the intensive care unit (ICU). There was no difference in the number of pulmonary hypertensive events between both groups: median (IQR); 2 (0-6,5) sildenafil, 1 (0-2) placebo, p=0,67. Neither inotrope score, time on the ventilator, ICU length of stay, nor fluid balance differed significantly. Urinary output was significantly lower in the sildenafil group.

Older age at the time of the Norwood procedure is a risk factor for early postoperative mortality†

The Norwood procedure is commonly used as first-step palliation in children with hypoplastic left heart syndrome or related congenital malformations. In most cases, the operation is carried out during the first week of life. Excessive pulmonary blood flow in univentricular physiology can soon lead to compromised cardiac function and pulmonary hypertension and could have an influence on postoperative outcomes. Between 1997 and 2011, 257 Norwood procedures were performed at the Kinderherz-Zentrum Linz. Twenty-seven patients were older than 20 days at the time of the Norwood operation without previous pulmonary artery banding. A retrospective analysis of the two age groups was carried out concerning early mortality, interstage mortality and major events until the bidirectional Glenn operation. Patients older than 20 days had a significantly higher mortality rate before the bidirectional Glenn operation than younger patients (44.4 vs 20.3%; P = 0.002). They also showed a significantly higher need for postoperative inhalative nitric oxide therapy (40.7 vs 14.5%, P = 0.002). Summarized risk for either successful postoperative cardiopulmonary resuscitation (CPR), extracorporeal membrane oxygenation therapy or mortality before the bidirectional Glenn operation was 59.3% in the older age group vs 23.8% in the younger age group (P < 0.001). Age older than 20 days seems to be an independent risk factor for early postoperative mortality and fatal events after the Norwood procedure. Long-persisting excessive pulmonary blood flow and preoperative cardiac decompensation can be the reason for postoperative lethal pulmonary hypertensive crisis and compromised ventricular function.

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.

Invited Commentary

Invited Commentary

Outcomes of Surgery for Simple Total Anomalous Pulmonary Venous Drainage in Neonates

Repair of total anomalous pulmonary venous drainage (TAPVD) in neonates remains a challenge as it is often associated with severe obstruction. We describe a large cohort of neonates who underwent TAPVD repair at a single institution. From 1973 to 2008, 112 patients underwent simple TAPVD surgery during the first month of life. Data collection occurred retrospectively. Preoperative pulmonary venous obstruction (PVO) occurred in 89 (79.5%) patients. There were 12 (10.7%) early deaths. Significant risk factors were bypass time greater than 65 minutes (p=0.014) and emergent surgery (p=0.005). Hospital mortality was unchanged throughout the 3 eras (1973 to 1988, 1989 to 1998, 1999 to 2008), despite an increase in patients with preoperative acidosis (p=0.004) and severe TAPVD obstruction (p=0.038) during the recent 10 years. There were 6 (6.25%) late deaths within 2 years of repair. Survival at 20 years was 83.4% (95% confidence interval 75 to 89). Risk factors for late death were operative weight 2.5 kg or less (p=0.004) and postoperative pulmonary hypertensive crisis (p=0.02). Reoperation for recurrent PVO was required in 13 patients (11.9%). Risk factors were operative weight 2.5 kg or less (p=0.035) and postoperative pulmonary hypertensive crisis (p=0.002). Follow-up was 96% complete and survivors (n=90) were asymptomatic at a median age of 11.7 years. Hospital mortality remained unchanged over the 36-year period. Survival beyond 2 years offers excellent outcome. Risk factors for mortality were the preoperative clinical status, prolonged bypass time, persisting micro-obstruction, and low operative weight. A reduction in mortality will likely require development of effective medical management for patients who have peripheral PVO not amenable to surgical repair.

Surgical Repair for D-Transposition of the Great Arteries Associated With an Aortopulmonary Window Using the Fenestrated Ventricular Septal Defect Patch as a Safety Adjunct

Transposition of the great arteries with a ventricular septal defect and an associated aortopulmonary window is a rare anatomic combination having a high risk for pulmonary hypertension. Arterial switch with closure of the ventricular septal defect and repair of the aortopulmonary window is the procedure of choice, but a postoperative pulmonary hypertensive crisis is a common occurrence associated with significant morbidity and mortality. This report describes one case of such an anatomic lesion, which was repaired successfully with a fenestrated ventricular septal defect patch as an adjunct to decrease the risk of a postoperative pulmonary hypertensive crisis.

The application of inhaled nitric oxide in management of postoperative pulmonary hypertension

Objective To evaluate the effects of inhaled nitric oxide (iNO) in management of postoperative pulmonary hypertension in the patients with congenital heart disease. Methods Thirty-two children with refractory pulmonary hypertension or pulmonary hypertensive crisis after open heart operations for congenital heart disease were treated with iNO. The changes of hemodynamics were observed. Results In iNO therapy group,the mean pulmonary artery pressure(mPAP) decreased from (38.0 ±3.2) mm Hg to (28.0 ±2.1) mm Hg,pulmonary vascular resistance (PVR) decreased from (62.2 ±6.7) kPa/(L·S) to (49.9 ±5.6) kPa/(L·S) ,PaO2/FiO2increased from (67.0 ±30. 1 ) mm Hg to (92. 6 ±25.0) mm Hg,and arterial oxygen saturation increased from 0. 78 ±0. 14 to 0. 84 ±0. 09 respectively. No toxic side effect was observed.Conclusion iNO is effective, safe to reduce pulmonary hypertension. It may play an important role in the treatment of postoperative pulmonary hypertension or pulmonary hypertensive crisis in the patients with congenital heart disease. Key words: Congenital heart disease; Nitric oxide; Pulmonary hypertension

An alternative technique for septation of the aortopulmonary window using a fenestrated, unidirectional valved fabric patch

Postoperative pulmonary hypertensive crises and right-sided heart failure remain the predominant cause of death in patients undergoing septation of the aortopulmonary window with moderate-to-severe pulmonary arterial hypertension (PAH). Although a unidirectional, fenestrated valved patch has been used in the setting of hypertensive atrial and ventricular septal defects to decompress the right-sided chambers in the event of pulmonary hypertensive crisis, there is no published report of aortopulmonary septation using this technique so far. We report the technique and results in 7 patients with a large aortopulmonary window with severe PAH using a fenestrated, unidirectional, valved polytetrafluoroethylene patch.

Aerosolized iloprost for postoperative pulmonary hypertensive crisis in children with congenital heart disease

Introduction Pulmonary hypertensive crisis (PHC) is a significant contributor to the morbidity and mortality of surgery for congenital heart defect. Management of such a potentially fatal complication has been evolving for the past decades. Inhaled iloprost has been reported as an alternative treatment for this condition. We evaluated the use of aerosolized iloprost as a rescue therapy for PHC in children undergoing congenital heart surgery. Methods In this clinical study, 12 high risk children were monitored in order to identify postoperative PHC after congenital heart repair. Factors being monitored included pulmonary artery pressure, systemic blood pressure, left atrial pressure, transcutaneous oximetry and heart rate. PHC was defined as an acute rise in pulmonary pressure which causes cardiopulmonary compromise as reflected by desaturation and hypotension. Despite conventional medical treatment to prevent postoperative PHC, children with PHC were therefore administered with aerosolized iloprost (0.5 μg/kg). Result Eight of the 12 children had one or more episodes of PHC, secondary to the pulmonary vasoreactivity. All responded to the aerosolized iloprost treatment, as demonstrated by a fall in their mean pulmonary pressure from 47.9 ± 14.9 to 30.2 ± 7.9 mmHg ( p = 0.012) and a rise in the arterial saturation from 82.2 ± 16.7 to 93.4 ± 11.5 % ( p = 0.012) while mean systemic blood pressure tended to increase from 59.4 ± 12.1 to 64 ± 10.3 mmHg ( p = 0.16). Conclusion In medical setting with limited access to the nitric oxide, inhaled iloprost is consider to be an effective alternative treatment for postoperative PHC in children undergoing congenital heart surgery.

The effect of inhaled nitric oxide and inhaled iloprost on hypoxaemia in a patient with pulmonary hypertension after pulmonary thrombarterectomy

Acute pulmonary hypertension with life-threatening right heart failure may complicate the postoperative course following cardiothoracic surgery. Both inhaled nitric oxide and inhaled iloprost, a stable analogue of prostacyclin, have been used frequently for this purpose in acute pulmonary hypertension of various origins. We present a case of a patient with acute pulmonary hypertension and severely impaired gas exchange following pulmonary thrombo-endarterectomy. Therapy with one inhaled vasodilator alone did not satisfactorily abort a postoperative pulmonary hypertensive crisis and low-output syndrome due to right heart failure. Combined inhaled nitric oxide and inhaled iloprost, however, showed additive effects. Hence, the combination of both drugs may be reasonable in cases where the standard therapy fails. The effect has been demonstrated by means of continuous blood gas monitoring.