Michael L. Ciccolo

Argatroban Anticoagulation for Cardiac Surgery with Cardiopulmonary Bypass in an Infant with Double Outlet Right Ventricle and a History of Heparin-induced Thrombocytopenia

Heparin-induced thrombocytopenia (HIT) is a life-threatening complication of heparin therapy. Anticoagulation in pediatric patients with HIT is challenging as there are no approved heparin substitutes. We report the use of the anticoagulant argatroban for cardiac surgery with cardiopulmonary bypass in an infant with double outlet right ventricle and a history of HIT.

MELD-XI Scores Correlate with Post-Fontan Hepatic Biopsy Fibrosis Scores

We tested the hypothesis that MELD-XI values correlated with hepatic total fibrosis scores obtained in 70 predominately stable, post-Fontan patients that underwent elective cardiac catheterization. We found a statistically significant correlation between MELD-XI values and total fibrosis scores (p = 0.003). Thus, serial MELD-XI values may be an additional useful clinical parameter for follow-up care in post-Fontan patients.

SPONTANEOUS TRACHEAL RUPTURE: A CASE REPORT

BACKGROUND Upper-airway disruption is a rare but potentially life-threatening phenomenon. It can occur spontaneously, be due to trauma, or be iatrogenically induced. Even more rare are such events reported in the pediatric population. OBJECTIVE This article discusses the presentation, diagnostic difficulties, and management of spontaneous tracheal rupture in a child. CASE REPORT A 3-year-old boy was brought by emergency medical services to our emergency department with a presumptive diagnosis of anaphylaxis. With progressive swelling and respiratory distress, the patient quickly deteriorated. He received i.v. epinephrine, chest compressions, and bag-valve mask ventilation. He was intubated without difficulty and with no noted airway edema. Concomitant bilateral needle thoracostomies were performed and subsequent bilateral tube thoracostomies were placed. Immediately after intubation and chest tube placements, the patients oxygen saturations and heart rate improved. Bronchoscopy failed to demonstrate any evident pathology. However, computed tomography scan revealed a defect in the posterior wall of the trachea proximal to the termination of the endotracheal tube. Cardiothoracic surgery was consulted and performed a primary repair of the tracheal defect. The patient was extubated soon after surgery, and he was discharged home neurologically intact. CONCLUSIONS The initial presentation of spontaneous tracheal rupture can be misleading and difficult to diagnose. After resuscitation, stabilization, and diagnosis, both surgical repair and nonoperative management have been reported as successful treatment measures for tracheal disruption.

Vascular Ring Diagnosis and Management: Notable Trends Over 25 Years.

Background: Vascular rings (VRs) are recognized as uncommon but not rare cardiovascular malformations. Methods: We analyzed data from all patients born in Southern Nevada, who underwent diagnosis and management of VR from 1990 to 2015, Results: From 1990 to 2015, a total of 92 patients were diagnosed prenatally and postnatally. Of the 92 patients, 73 (79%) had right aortic arch and aberrant left subclavian artery (RAA-ALS) with a left ductus arteriosus or ligamentum, 17 (19%) had a double aortic arch (DAA), and 2 (2%) had a pulmonary artery sling. Of the 92 patients, 75 had an isolated VR and 17 VR had significant additional congenital heart disease (CHD). Of the 75 patients with an isolated VR, 52 underwent surgical repair, and the most common surgical diagnosis was DAA in 6 (66%) of 9 for the period 1990 to 2005 versus less common in 9 (21%) of 43 during the period 2006 to 2015, P < .05. The isolated VR repair age significantly negatively correlated with increasing time from 1990 to 2015, R = −0.7 (P < .0001). Of the 75 isolated VR, 23 remain asymptomatic. The 17 VR with CHD were treated during infant palliation or intracardiac repair. Of the total 92 VR, 60 were born after a 2004 community introduction of the three-vessel fetal echocardiography view, from then the prenatal-detection rate has significantly increased—2004 to 2006, 0 (0%) of 9; 2007 to 2009, 1 (9%) of 11; 2010 to 2012, 11 (55%) of 20; and 2013 to 1015, 14 (70%) of 20 (P < .0001). Conclusion: Over 25 years in Southern Nevada, VR prenatal diagnosis has increased, isolated VR age at surgery has decreased, and the percentage of those with RAA-ALS has increased.

Modified De Vega Mitral Valve Annuloplasty in an Infant With Endocarditis

Surgical management of infant mitral valve regurgitation is challenging. There are no large surgical series of mitral valve repair in infants. Mitral valve repair is preferable to replacement. We report the modified De Vega adjustable annuloplasty with mitral valve repair in an infant with mitral valve regurgitation from endocarditis.

All Cardiac Right Ventricular Outpouches Are Not Created Equal

Congenital right ventricular diverticula and aneurysms are rare. Clinically, a diverticulum is difficult to distinguish from an aneurysm. Four diverse right ventricular cameral defects or right ventricular outpouches (RVOs) are described together with the management of each. Surgery may be necessary if an RVO has thin walls. However, if an RVO is composed of uniform thick contractile walls, conservative follow-up care likely is appropriate.

An Observation from Liver Biopsies Two Decades Post-Fontan

This brief report describes an observation from liver biopsy results in nonfailing Fontan patients, currently in their second postoperative decade. In three patients, with either atriopulmonary or atrioventricular connections and functional left ventricles, we found no portal fibrosis. In contrast, we found portal fibrosis in three clinically similar, nonfailing Fontan patients with lateral tunnel connections and functional left ventricles. We recognize the results may be secondary to chance; nevertheless, we speculate about possible relevancy.

A composite noninvasive index correlates with liver fibrosis scores in post-Fontan patients: Preliminary findings

OBJECTIVE We hypothesized that clinic-based, hepatic-ultrasound, elastography measurements, either alone or in combination with other noninvasive variables, might correlate with liver-biopsy fibrosis scores in patients post-Fontan. METHODS Between March 2012 and February 2017, we identified patients post-Fontan that underwent elective cardiac catheterization and simultaneous transvenous hepatic biopsy. From this group, we selected patients that met inclusion criteria for liver-ultrasound, shear-wave elastography. Utilizing the results of elastography, laboratory testing, and time post-Fontan, we constructed a composite Fontan hepatic index as a sum of elastography measurements in kilopascals, model for end-stage liver disease excluding INR scores, and the square root of the number of years post-Fontan. Further, we analyzed correlations between Fontan hepatic index values and fibrosis scores from hepatic biopsy. RESULTS We identified a total of 79 post-Fontan patients that underwent cardiac catheterization and liver biopsy. Of the 79 patients, 53 met inclusion criteria, and 32 consented to undergo hepatic-ultrasound elastography. Of the 32 that underwent elastography, data from 30 patients was used for analysis. We found no statistically significant differences in demographics, laboratory values, or cardiac catheterization data between the 30 included patients and the 21 that did not participate. Utilizing data from the 30 included patients, we found a strong, highly statistically significant correlation between the Fontan hepatic index values and total fibrosis scores (R = 0.8, P < .00001). However, the cohort size prevented reliable discriminating cut-off values for the range of total fibrosis scores. CONCLUSIONS In a small cohort of patients post-Fontan, preliminary findings suggest that the composite Fontan hepatic index might be a clinically useful, noninvasive method of serially monitoring for hepatic fibrosis. Further studies, with large patient cohorts, are necessary to validate our findings and develop clinically useful discriminatory cutoff values.

Right aortic arch with situs solitus frequently heralds a vascular ring

OBJECTIVE We hypothesized that a right aortic arch in situs solitus, with or without an associated cardiovascular malformation, is often associated with a vascular ring. METHODS From those born in Southern Nevada between March 2012 and March 2017, we identified 50 (3.6 per 10,000 live births) with a right aortic arch and situs solitus. From the 50 patients, 6 did not meet inclusion criteria for further analysis. RESULTS Of the 44 remaining, 33 (75%) had a vascular ring. Of the 33 with a vascular ring, 26 (79%) occurred with an isolated right aortic arch, and 7 (21%) had an associated cardiovascular malformation. Of the total 44 patients with a right aortic arch in situs solitus, 34 (79%) were diagnosed prenatally. CONCLUSIONS In conclusion, we found a right aortic arch in situs solitus was often associated with a vascular ring. Further, to the best of our knowledge, no previous general population study has demonstrated an equal or higher right aortic arch, prenatal detection rate of 79%.

An Increased Incidence of Total Anomalous Pulmonary Venous Connection among Hispanics in Southern Nevada

OBJECTIVE Ethnicity may influence the occurrence of specific cardiac malformations. DESIGN We retrospectively analyzed the occurrence of isolated total anomalous pulmonary venous connection in the Hispanic and non-Hispanic populations of Southern Nevada over a 10-year period from 2003 to 2013. RESULTS The mean cases per 100,000 live births among Hispanics was 19.8 (99% confidence interval 5.9-33.7) and among non-Hispanics was 2.5 (99% confidence interval 0.4-4.6), P = 0.007. CONCLUSIONS We found a significantly higher incidence of isolated total anomalous pulmonary venous connection in Hispanics vs. non-Hispanics.