World Journal for Pediatric and Congenital Heart Surgery

Truncus arteriosus is a complex and heterogeneous form of congenital heart defect. Many of the risk factors from several decades ago, including late repair and interrupted aortic arch, have been mitigated through better understanding of the entity and improved surgical techniques. However, truncal valve dysfunction remains an important cause of morbidity and mortality. The purpose of this study was to evaluate the anatomic factors associated with truncal valve dysfunction and the need for truncal valve surgery.

This was a retrospective review of 72 infants who underwent repair of truncus arteriosus at our institution. The median age at surgery was nine days, and the median weight was 3.1 kg. Preoperative assessment of truncal valve insufficiency by echocardiography revealed no or trace insufficiency in 30, mild in 25, moderate in 10, and severe in 7. The need for truncal valve surgery was dictated by the severity of truncal valve insufficiency.

Sixteen (22%) of the 72 patients undergoing truncus arteriosus repair had concomitant truncal valve surgery. Anatomic factors associated with the need for truncal valve surgery included an abnormal number of truncal valve cusps (P < .005), presence of valve dysplasia (P < .005), and the presence of an anomalous coronary artery pattern (P < .005). Fifteen (94%) of the sixteen patients who underwent concomitant surgery had two or all three of these anatomic factors (sensitivity = 94%, specificity = 85%).

This study demonstrates that the presence of specific anatomic factors was closely associated with the presence of truncal valve insufficiency and the need for concomitant truncal valve surgery. Preoperative evaluation of these anatomic factors may provide a useful tool in determining who should undergo concomitant truncal valve surgery.

Congenital tracheal stenosis (CTS) is a serious and rare condition that often presents a complex surgical challenge. We reviewed the evolution of surgical approaches to the management of CTS at a single institution.

We conducted a retrospective clinical review of all patients who underwent surgical repair of CTS at our institution. From January 1992 to January 2014, a total of 30 patients (median age 92 days; range 3 days-1.7 years) underwent surgery for CTS at our institution. Techniques included slide tracheoplasty (n = 16), tracheal resection (n = 10), pericardial patch tracheoplasty (n = 3), and costal cartilage tracheoplasty (n = 1). Twenty-two (73%) patients had associated intracardiac or great vessel anomalies, including 18 (60%) with left pulmonary artery sling. Five (17%) patients had associated single lung malformation. All procedures were performed via a median sternotomy with cardiopulmonary bypass.

Median postoperative length of stay was 25 days (range, 5-431 days). Late airway reintervention was required in seven (26%), including two (15%) infants after slide tracheoplasty, two (22%) posttracheal resection, and two (100%) post pericardial patch tracheoplasty. The one patient who underwent costal cartilage tracheoplasty required multiple reinterventions. Overall mortality was 13% (n = 4), two deaths occurred post slide tracheoplasty, one death occurred after tracheal resection, and one after pericardial patch tracheoplasty, respectively.

Slide tracheoplasty is the procedure of choice for repair of CTS, while tracheal resection is a viable option for patients with discreet, short-segment stenosis.

Little is known about practitioner preference, the availability of technology, and variability in practice with respect to hemodynamic monitoring and vasoactive drug use after congenital heart surgery. The aim of this study was to characterize current hospital practices related to the management of low cardiac output syndrome (LCOS) across Italy.

We issued a 22-item questionnaire to 14 Italian hospitals performing pediatric cardiac surgery.

Electrocardiogram, invasive blood pressure, central venous pressure, pulse oximetry, diuresis, body temperature, arterial lactate, and blood gas analysis were identified as routine in hemodynamic monitoring. With regard to advanced hemodynamic monitoring, pulmonary arterial catheter and transpulmonary thermodilution were available in 43% of the centers, uncalibrated pulse contour methods in 29% of the centers, and transesophageal/transthoracic echocardiograms in all of the centers. Dopamine added to milrinone was the most frequent drug regimen for LCOS prevention after cardiopulmonary bypass. Overall, 86% of centers used milrinone alone as the initial treatment for LCOS with elevated systemic vascular resistances and levosimendan, the second preferred choice. In cases of LCOS with low vascular resistance, epinephrine was the first choice (10 centers), dopamine was the second choice (4 centers), followed by vasopressin and norepinephrine (3 centers). For treatment of LCOS with elevated pulmonary resistances, milrinone was the first choice (eight centers), followed by inhaled nitric oxide (five centers).

The survey shows that advanced hemodynamic monitoring is rarely performed. The most commonly used vasoactive drugs are milrinone, levosimendan, dopamine, epinephrine, vasopressin, and norepinephrine. Guidelines on the topic are warranted.

Literature on the use of nicardipine, a dihydropyridine calcium channel antagonist, in children recovering from cardiac surgery is sparse and, to our knowledge, nonexistent in children with single ventricle anatomy. We aimed to report our experience with nicardipine in these patient populations.

We performed a retrospective review of children recovering from aortic coarctectomy or superior cavopulmonary anastomoses who received nicardipine for hypertension at our institution between 2007 and 2013. Hemodynamic variables prior to and after nicardipine initiation were compared using paired t tests.

Seven children recovering from aortic coarctectomy (median age 8.6 months, range: 1.5 months-7.9 years) and four children recovering from superior cavopulmonary anastomosis (median age: seven months, range: five-nine months) were reviewed. For all patients, at six hours after initiation of nicardipine, mean systolic blood pressure was significantly decreased, 123 ± 19 versus 103 ± 14 mm Hg (P = .001), as were diastolic blood pressure, 68 ± 20 versus 53.5 ± 10 mm Hg (P = .041), and sodium nitroprusside dose, 4.3 ± 2.9 versus 1.3 ± 1.7 mcg/kg/min (P = .002). Further, within 24 hours, serum lactate decreased from 1.45 ± 0.82 to 0.81 ± 0.29 mg/dL (P = .016). Heart rate, blood urea nitrogen, and serum creatinine measurements were statistically unchanged.

Nicardipine effectively decreased blood pressure without apparent adverse events in a small cohort of children with postoperative hypertension while recovering from aortic coarctectomy or superior cavopulmonary anastomosis. Further research comparing nicardipine to more conventional titratable antihypertensive agents in these patient populations is warranted.

Patients who have undergone a previous bidirectional superior cavopulmonary anastomosis and have hypoxemia and anatomy considered unsuitable for the Fontan completion present a difficult subset. We performed common carotid artery-to-internal jugular vein (CCA-IJV) shunt in these patients.

Between January 2010 and January 2015, eight patients underwent a CCA-IJV shunt. Their records were analyzed retrospectively.

There were no early deaths. The baseline arterial saturation prior to the shunt procedure was median 67% (range 60%-72%), which increased to median 83% (range 80%-90%) after the procedure. The preoperative arterial oxygen partial pressure (Pao ₂) ranged from 30 to 49 mm Hg (median 40 mm Hg). The hematocrit dropped from a preoperative median of 65% (range 55%-72%) to a median of 45% (range 42%-48%) after the procedure. Median intensive care unit stay was 2.5 days, and the median hospital stay was 7 days. Median follow-up was 24 months. At the last follow-up, these patients were classified in New York Heart Association class II.

In a selected group of patients who are considered to be unsuitable for completion of Fontan circulation following superior cavopulmonary anastomosis, creation of a CCA-IJV shunt may be helpful to improve saturations and improve ventricular function.

Prolonged pleural effusion following Fontan operation is common and increases morbidity and hospital length of stay. Vasopressin (VP), a neurohypophysial hormone, has numerous effects on the cardiovascular system. The most notable is increased peripheral vascular resistance, but it may also reduce capillary leakage by tightening endothelial intercellular junctions and reducing capillary hydrostatic pressure We reviewed our experience with the perioperative administration of VP following Fontan operation.

We retrospectively reviewed the records of 62 consecutive patients who underwent Fontan operation from January 2004 to June 2014. In January 2010, VP was introduced as part of the standard perioperative management of patients undergoing Fontan operation at our center. For this retrospective observational study, patients were grouped according to the use (VP; N = 40) or nonuse (non-VP; N = 22) of VP (0.3-0.5 mU/kg/min) in the perioperative period. The primary end point analyzed was chest tube output. Secondary end points analyzed included fluid balance and length of hospital stay, with groups compared using Mann-Whitney U test.

There was no hospital mortality. Median total chest tube output was 22 mL/kg in the VP group and 68 mL/kg in the non-VP group (P < .001). The median total duration of chest tube indwelling time was five days in the VP group and was 11 days in the non-VP group (P < .001). Median fluid balance on first postoperative day was 13 and 38 mL/kg, respectively (P < .001). Median hospital stay for VP and non-VP groups was 9 and 16 days, respectively (P = .002).

The more recent group of patients undergoing Fontan operations, all of whom received VP perioperatively, had less chest tube output and shorter duration of chest tube drainage after the Fontan operation relative to the earlier patient group whose perioperative management did not include VP. They also experienced less positive fluid balance in the early postoperative period and shorter hospital length of stay than the patients from the earlier era.

Heterotaxy has been demonstrated to reduce survival. There are several different subgroups of patients, however, and no single study has had a large number of patients and analyzed survival across the different subgroups such as patients born in different eras, patients with right and left isomerism, and patients with biventricular or functionally univentricular hearts. This study pools previously reported data from Kaplan-Meier curves and performs such subgroup analysis.

A systematic review of the literature was performed to identify studies reporting survival of patients with the so-called “heterotaxy” by means of Kaplan-Meier survival curves. Data were extracted from these survival curves and then pooled together. A polynomial regression was then used to generate a pooled survival curve. This was done for all patients, those born in a more recent era, those with right and left isomerism, and those with biventricular or functionally univentricular hearts.

Those born in the more recent era (after 2000) had increased survival compared to the overall cohort. Those with left isomerism tended to have a survival benefit compared to those with right isomerism until about 16 years of age, beyond which those with right isomerism developed a survival benefit. Those with biventricular hearts had a survival benefit compared to those with left isomerism.

Survival in the so-called heterotaxy syndrome is based on several factors, which include era of birth, sidedness of isomerism, and whether the heart is biventricular or functionally univentricular.