Update on Perioperative Pediatric Pulmonary Hypertension Management.

Pediatric pulmonary hypertension is a disease that has many etiologies and can present anytime during childhood. Its newly revised hemodynamic definition follows that of adult pulmonary hypertension: a mean pulmonary artery pressure >20 mmHg. However, the pediatric definition stipulates that the elevated pressure must be present after the age of three months. The definition encompasses many different etiologies, and diagnosis often involves a combination of noninvasive and invasive testing. Treatment often is extrapolated from adult studies or based on expert opinion. Moreover, although general anesthesia may be required for pediatric patients with pulmonary hypertension, it poses certain risks. A thoughtful, multidisciplinary approach is needed to deliver excellent perioperative care.

Perioperative management and considerations in pediatric patients with connective tissue disorders undergoing cardiac surgery.

BACKGROUND: Marfan syndrome and Loeys-Dietz syndrome are connective tissue disorders associated with cardiac and vascular disease. Patients often require surgical repair, but limited data exist to describe their perioperative management. AIMS: Our goals were to review the perioperative features of patients with Marfan and Loeys-Dietz syndrome that may affect anesthesia care and to describe the differences in preoperative clinical characteristics and intra-operative anesthetic management. METHODS: We conducted a retrospective cohort study of patients with Marfan and Loeys-Dietz syndrome who underwent cardiac surgery at a single institution. We collected demographic and perioperative data from the electronic medical record and performed descriptive statistics to characterize the patient populations and describe their anesthetic management. RESULTS: In 71 patients (40 Marfan, 31 Loeys-Dietz), we found significant differences between the Marfan and Loeys-Dietz patients in airway difficulty, preoperative weight, blood utilization, valvular disease, and age at first cardiac surgery. Patients with Loeys-Dietz syndrome had higher preoperative rates of severe noncardiac comorbidities, including gastroesophageal reflux and asthma that required chronic medical therapy. CONCLUSIONS: Despite undergoing similar surgical procedures, patients with Marfan and Loeys-Dietz syndrome have different intrinsic patient characteristics and comorbidities that may affect their perioperative care. This retrospective cohort study identified some factors, but additional collection and reporting of patient data based on multicenter experience are essential for the ongoing optimization of perioperative care in these patient populations.

Acute Renal and Hepatic Failure in an Adolescent: An Unusual Presentation of Multiple Aortic Aneurysms.

Aortic dissection secondary to thoracoabdominal aortic aneurysms is very uncommon in children, and this life-threatening diagnosis requires a high clinical index of suspicion. Unlike adults, in whom atherosclerosis, inflammation, and advanced age are typically contributing factors, aortic dissection in children is usually due to nonatherosclerotic causes.Aortic aneurysms can be asymptomatic when small but, when significantly enlarged, can compromise organ function and dissect, resulting in high mortality rates. It is therefore critical that children with this uncommon condition be identified early when medical or surgical management can potentially improve outcome. We describe a 15-year-old patient with multiple aortic aneurysms with dissection whose presentation includes chronic anemia, acute-on-chronic renal failure with hyperkalemia, and liver injury.

Flecainide Toxicity Secondary to Accidental Overdose: A Pediatric Case Report of Two Brothers.

Flecainide is a class 1C antiarrhythmic with a narrow therapeutic window and thereby a high-risk medication for causing acute toxicity. Dysrhythmias secondary to flecainide ingestion are often refractory to antiarrhythmics and cardioversion, and patients commonly require extracorporeal support. We review the successful resuscitation of two brothers aged 2 and 4 who presented two years apart with unstable wide-complex tachyarrhythmia suspicious for severe flecainide toxicity. Each patient received sodium bicarbonate and 20% intravenous lipid emulsion with a full recovery. While extracorporeal support is often required following flecainide ingestion, we present two cases where it was avoided due to aggressive multimodal management with sodium bicarbonate, electrolyte repletion, and 20% intravenous lipid emulsion. In addition, avoidance of agitation-induced tachycardia may be beneficial.

Membrane Oxygenator Use With Biventricular Assist Device: Facilitation of Support and Lung Recovery.

We present the use of a low-resistance membrane oxygenator (Quadrox D, Maquet) in series with a pulsatile right ventricular assist device (Berlin Heart EXCOR, Berlin Heart) in a patient with biventricular support who required high-frequency oscillatory ventilation (HFOV), due to refractory acute respiratory distress syndrome associated with Cytomegalovirus pneumonia. The high mean airway pressure associated with the use of HFOV resulted in a significant negative impact on left ventricular assist device (LVAD) filling that led to a combined respiratory and metabolic acidosis and the need for vasopressor support. Oxygenator placement enabled transition to conventional ventilation and the discontinuation of vasopressor support. This case demonstrates the feasibility and safety of the use of this lung support system in patients requiring ventricular assist device (VAD) support.