Anesthetic management for percutaneous reverse Potts shunt creation in children with refractory idiopathic pulmonary arterial hypertension: A case series.

Percutaneous reverse Potts shunt improves right ventricular function in patients with suprasystemic idiopathic pulmonary arterial hypertension. There are no data regarding the anesthesia in this high-risk procedure. We report our experience of the anesthetic management for the creation of percutaneous reverse Potts shunt in children with suprasystemic idiopathic pulmonary arterial hypertension. This study included 10 patients presenting with symptomatic idiopathic pulmonary arterial hypertension despite undergoing medical treatment. All patients underwent gradual induction of anesthesia to maintain hemodynamic stability (etomidate, n = 8; ketamine, n = 4). Four patients needed extracorporeal life support: 2 were rescued after cardiac arrest and 2 had elective extracorporeal life support due to preprocedural dysfunctional right ventricle and/or small left ventricle volumes with reduced cardiac output. All patients were admitted to the pediatric cardiac intensive care unit (4 [2-5] days). All patients with extracorporeal life support died. None of the six survivors needed pulmonary transplantation. Both ketamine and etomidate support hemodynamics. High-dose opioid technique has the advantage of blunting noxious stimuli and subsequent increase in pulmonary vascular resistance. We recommend using multimodal monitoring with transesophageal echocardiography. The 100% mortality of extracorporeal life support patients, probably too sick to undergo such procedure, may question its usefulness. Further studies should identify suitable candidates for percutaneous reverse Potts shunt creation.

A case series of transcatheter Potts Shunt creation in a pediatric population affected with refractory pulmonary artery hypertension: focus on the role of ECMO.

PURPOSE: Patients with suprasystemic idiopathic pulmonary hypertension (S-PAH) have a poor prognosis. Therapeutic options are limited. Reverse Potts shunt creation modifies physiology transforming patients with PAH into Eisenmenger physiology with a better outcome. Percutaneous transcatheter stent secured aortopulmonary connection (transcatheter Potts Shunt, TPS) is a feasible very high-risk procedural option in such patients. We report our experience with patients undergoing TPS at our institution requiring extracorporeal membrane oxygenation (ECMO) support. METHODS: A prospective observational study of patients with drug-refractory PAH, worsening NYHA class, and right ventricular failure undergoing TPS. Two patients required rescue ECMO for cardiac arrest during the procedure. Subsequently, "standby ECMO" was available in all the following cases and elective support was provided in patients with extremely poor conditions. RESULTS: Ten pediatric patients, underwent TPS at our institution. Two patients were rescued by ECMO after cardiac arrest during the shunt creation. This occurred as a result of the acute loading of the left ventricle (LV) after retrograde aortic arch filling through the Potts shunt. Following this, another two patients underwent elective ECMO after the uneventful induction of anesthesia. They all died postoperatively despite a successful TPS procedure. The causes of death were not related to the use of ECMO, but the complication of severe PAH. Six patients with successful TPS did not require ECMO and survived. CONCLUSIONS: TPS is a pioneering procedure offering the opportunity to treat high-risk idiopathic drug-refractory PAH patients. Acute LV failure is a complication of TPS in patients with S-PAH. Elective ECMO, an option to avoid circulatory arrest and acute profound hypoxia secondary to exclusive right-to left shunt systemic perfusion by Potts shunt and LV dysfunction with resulting pulmonary edema, may be used at the early stage of the learning curve, but it does not influence the prognosis of these patients which remains poor.

Intraoperative hypoxia secondary to pneumothorax: The role of lung ultrasound.

Intraoperative pneumothorax during general anesthesia is a dangerous event. It is a possible cause of sudden intraoperative hypoxia, which can be critical especially in high-risk patients such as those with end-stage heart failure. Early diagnosis and effective treatment are essential. We describe the case of a pneumothorax during cardiac transplantation, diagnosed by ultrasound and immediately treated. A good skill in lung ultrasound is advantageous in the management of intraoperative hypoxia, particularly for prompt diagnosis of pneumothorax.

Use of a biodegradable, oversized stent in a child with tracheomalacia secondary to vascular external compression.

We describe the implantation of an absorbable, custom-made stent of polydioxanone to treat tracheomalacia in a 5-month-old patient with extrinsic compression by a double aortic arch. The use of an absorbable, oversized stent treated the tracheal collapse caused by vascular compression, avoided removal procedures, and allowed the infant's growth. The use of an oversized stent prevented stent migration and gave minimal problems of granulation.

Chronic Kidney Disease in Adolescents after Surgery for Congenital Heart Disease.

BACKGROUND: The onset of chronic kidney disease (CKD) is an important prognostic factor in young adults with congenital heart disease (CHD). Although it is likely that CKD is manifest early in CHD patients, the prevalence among adolescents is still unknown. The National Kidney Foundation's Kidney Disease Improving Global Outcomes guidelines 2012 recommend new equations for the estimated glomerular filtration rate (eGFR) and highlight the importance of albuminuria for CKD screening. The objective of the present study was to estimate the prevalence of CKD in CHD adolescents. METHODS: This observational cross-sectional study included 115 patients aged 10-18 years attending the cardiologic outpatient clinic at our institution as a follow-up after cardiac surgery in infancy related to various CHDs. CKD assessment used the CKD criteria 2012, including eGFR equations based on serum creatinine and cystatin C, and measurement of albuminuria. RESULTS: No patient had an eGFR <60 mL min-1 1.73 m-2. However, 28.7% of all patients (95% CI 20.7-37.9) had eGFRbetween 60 and 89 mL min-1 1.73 m-2 when estimated by the bedside Schwartz creatinine-based equation,and 17.4% (95% CI 11.2-24.1) had eGFRbetween 60 and 89 mL min-1 1.73 m-2 when estimated by the Zappitelli equation, combining creatinine and cystatin C. Of all patients, 20.0% (95% CI 12.1-26.7) had orthostatic proteinuria, and none had persistent albuminuria. CONCLUSIONS: There was no evidence of CKD in the present population aged 10-18 years. The significance of an eGFR between 60 and 90 mL min-1 1.73 m-2 is not concordant for this age range and requires further investigations.

Somatic and cerebral near infrared spectroscopy for the monitoring of perfusion during neonatal cardiopulmonary bypass.

OBJECTIVES: The somatic-cerebral regional oxygen saturation (rSO2) gradient has the potential to reveal a low cardiac output state at an early stage, when systemic vascular resistance increases in order to maintain cerebral perfusion, and results in a drop in somatic rSO2. We hypothesized that during neonatal cardiopulmonary bypass (CPB), the somatic-cerebral rSO2 gradient can also signal poor perfusion, as assessed by an increase in lactate concentration. METHODS: In a retrospective review of neonatal bypass records, we investigated the association between the somatic-cerebral rSO2 gradient and serial lactate measurements. The somatic-cerebral rSO2 gradient, i.e. the area between the somatic and cerebral rSO2, was calculated for each CPB time interval between 2 lactate measurements, using the trapezoidal method. The association between the somatic-cerebral rSO2 gradient and lactate variation was explored using a mixed model for repeated measurements adjusted for the CPB temperature across intervals. RESULTS: A total of 94 CPB intervals were analysed in 24 neonates, median age 6 days, median weight 3.1 kg. The median lactate variation per interval was +0.2 mmol l-1 (range -1.5 to +2.7), the median somatic-cerebral gradient was 0 (range -1229% min to +2049% min) and the median CPB temperature was 29.5°C (IQR 28.1-33.4). There was an independent association between the lactate increase and the somatic-cerebral rSO2 gradient (regression coefficient = -0.0006 ± 0.0002, P = 0.009). CONCLUSIONS: A decrease in the somatic-cerebral rSO2 gradient was associated with an increase in lactate concentration, suggesting that the somatic-cerebral rSO2 gradient is a useful monitoring tool for the adequacy of perfusion during neonatal CPB.

Hemodynamic effects of levobupivacaine after pediatric caudal anesthesia evaluated by transesophageal doppler.

BACKGROUND: The aim of this study is to determine if there are significant differences in hemodynamic effects between combined general-regional anesthesia using levobupivacaine 0.25% 2 ml x kg(-1) via the caudal route in comparison with balanced general anesthesia using continuous infusion of remifentanil in young children undergoing genitourinary surgery. PATIENTS AND METHODS: 62 ASA I-II pediatric patients (12 female, 50 male) aged 6 months to 7 years undergoing genitourinary surgery were included in the study. Patients were randomly allocated into one of two groups of 31 patients each. Group Caud received caudal blockade with levobupivacaine 0.25% 2 mg x kg(-1) in combined general-regional anesthesia and Group Gen received balanced general anesthesia with remifentanil. The noninvasive hemodynamic parameters were measured in each group 5 min after induction of general anesthesia or caudal block (Tcaud), after further 5 min coincident with skin incision (Tsi), 10 min after skin incision (T10i), at the end of surgical procedure (Tend). The time between Tcaud and Tsi was 10 min and the measurements during the interval time T10i-Tend were performed every 15 min according to the duration of surgical procedures. RESULTS: There was a decrease in all measured hemodynamic parameters at skin incision. The decreases occurred in both groups with those in the caudal group occurring at skin incision and those in the balanced anesthesia group occurring at 10 min after skin incision. These variations showed no significant differences for any of the stated outcomes; neither between the groups at each time point nor in the caudal in comparison with baseline measurements. CONCLUSIONS: Using transesophageal Doppler no differences in hemodynamic parameters could be detected between balanced general anesthesia with either caudal levobupivacaine or remifentanil infusion. Both techniques showed good hemodynamic stability with only minor changes from baseline over time which are unlikely to be of clinical significance except possibly in patients with preexisting cardiovascular compromise. Other studies with noninvasive monitoring in a larger population are required to better understand the consequences of caudal blockade on CO and on regional blood flow in infants.

Safety and Feasibility of the Transcatheter Approach to Create a Reverse Potts Shunt in Children With Idiopathic Pulmonary Arterial Hypertension.

BACKGROUND: The reversed Potts shunt improves right ventricular (RV) function in patients with suprasystemic pulmonary arterial hypertension (PAH). The proximity of the left pulmonary artery (LPA) to the descending aorta (DAo) permits the creation of a transcatheter connection. We sought to assess the safety, feasibility, and hemodynamic efficacy of the transcatheter Potts shunt (TPS) in children. METHODS: The TPS procedure was performed using radiofrequency energy for vessel perforation and deployment of a covered stent to connect the DAo and LPA. Procedural details and clinical follow-up data were collected prospectively. RESULTS: A TPS was successfully created in 6 children (mean age, 11.0 ± 4.2 years) with drug-refractory suprasystemic PAH and deteriorating RV function. All patients exhibited nearly complete equalization of aortic and pulmonary pressures and improvement in RV contractility within days after TPS placement. Two patients with pre-existing severe biventricular dysfunction and pericardial effusion experienced acute low-output states immediately after shunt creation because of sudden reductions in left ventricular (LV) preload, resulting in cardiac arrest, irreversible brain damage, and death. Stent dislodgement and embolization into the iliac artery occurred in 1 patient. The stent was successfully secured and followed by placement of a second stent at the target location. The procedure was uncomplicated in 4 patients, who remain alive after a mean follow-up of 10 ± 2.6 months. Intravenous vasodilator therapy was weaned uneventfully after TPS in 3 patients. CONCLUSIONS: TPS creation in children is feasible and results in hemodynamic improvement. Further insights into high-risk markers, such as reduced preprocedural LV function and preload reserves, are important for guiding patient selection.

Postoperative B-type natriuretic peptide monitoring for the assessment of the magnitude of shunting through Blalock-Taussig anastomoses.

BACKGROUND: The Modified Blalock-Taussig shunt (MBTS) is the most common palliative operation performed in patients with complex cardiac defects. Postoperative morbidity and mortality rates are high, mainly due to shunt thrombosis and over-shunting. Over-shunting may be difficult to identify postoperatively based on conventional criteria. Since plasma B-type natriuretic peptide (BNP) concentrations correlate with the magnitude of shunting in various left-to-right shunt cardiac defects, we investigated its ability to identify postoperative MBTS over-shunting. METHODS AND RESULTS: This retrospective, observational study included 42 consecutive patients (median age 9.50days, IQR: 6.00-58.25) undergoing MBTS for obstruction of the pulmonary blood flow at a tertiary referral pediatric cardiac center. The BNP threshold concentrations which accurately predicted outcome and MBTS over-shunting were derived using the ROC methodology. 443 BNP concentrations were analysed. The presence of atrio-ventricular valve regurgitation was the most important component of overall variance (72.75%). In 34 patients without regurgitation, BNP concentrations were predictive of a duration of mechanical ventilation >8days and of intensive care stay >11days, with ROC areas of 0.655 [0.597-0.719], 0.650 [0.589-0.711], a negative predictive value for the >1035pgmL-1 threshold of 0.93 and 0.96 respectively. SaO2 was less accurate for the prediction of both outcomes. In patients in whom the pulmonary flow was entirely MBTS-supplied, a BNP concentrations >1052pgmL-1 was predictive of a pulmonary-to-systemic ratio>2. CONCLUSION: In MBTS patients without atrio-ventricular valve regurgitation, maintaining BNP below 1000pgmL-1 may represent a therapeutic target to avoid over-shunting.