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Background Aortic valve replacement in children represents an important challenge. Concerns regarding pulmonary autograft and homograft longevity requiring reoperations are well recognized. Very long-term outcomes after the Ross procedure are still unknown. We reviewed our experience with the Ross procedure, aiming to define very long-term survival rate and freedom from reintervention. Methods This was a single-center retrospective cohort including 63 consecutive children who underwent the Ross procedure. Median follow-up duration was 20.5 years. Time-related events were assessed using Kaplan-Meier estimator. Results There were 51 (81%) boys, mean age 10.1 ± 5.8 years. Isolated aortic stenosis was the most common diagnosis (n = 29, 46%) and 34 (54%) patients previously underwent cardiac surgery. There was 1 (1.6%) in-hospital death. Overall survival at 5, 15, and 25 years was 96.7%, 94.4%, and 94.4%, respectively. Freedom from any autograft-related reintervention was 98.1%, 86.4%, and 61.2% at 5, 15, and 25 years, respectively. Fifteen (24%) patients underwent autograft reoperations. Among them, 10 (67%) patients underwent valve-sparing autograft reoperation. Freedom from any pulmonary conduit reintervention was 93.2%, 58.2%, and 28.3% at 5, 15, and 25 years, respectively. Thirty (46.6%) patients underwent conduit reintervention (8 percutaneous, 22 surgical replacements). Conclusions The pediatric Ross procedure is associated with excellent long-term survival. Ross-related reinterventions are more than twice as common on the pulmonary homograft than on the autograft.

Fibrin sheath formation around long-term indwelling central venous catheters is common and usually benign. Fibrin sheath can persist after catheter removal and rarely leads to complications. This is a report of three pediatric oncology patients that required cardiac surgery for cardiac embolization of a "ghost" catheter several years after catheter removal. One case required tricuspid valve replacement for complete tricuspid valve destruction and two had erosion through the atrial wall. The severity of these rare complications mandates follow-up of "ghost" catheters in pediatric oncology patients.

This cohort study compares the long-term outcomes associated with valve-sparing procedures vs transannular patches for correction of tetralogy of Fallot., Key Points Question Do valve-sparing (VS) operations have better long-term outcomes than transannular patches (TAP) for correction of tetralogy of Fallot (TOF) when stratifying for residual lesions after surgical treatment? Findings In this cohort study of 528 patients with repaired TOF in the province of Quebec, Canada, from 1980 to 2015, individuals who underwent a VS procedure had statistically significantly increased 30-year survival compared with individuals who underwent TAP. Moreover, individuals who underwent a VS procedure had statistically significantly fewer reinterventions, even in the presence of moderate residual pulmonary stenosis. Meaning These findings suggest that it is beneficial to perform a VS procedure for correction of TOF, even in the presence of moderate residual stenosis, compared with TAP repair., Importance The choice of the right surgical technique for correction of tetralogy of Fallot (TOF) is contentious for patients with a moderate to severe right outflow tract obstruction. The use of a transannular patch (TAP) exposes patients to chronic pulmonary regurgitation, while valve-sparing (VS) procedures may incompletely relieve pulmonary obstruction. Objective To compare 30-year outcomes of TOF repair after a VS procedure vs TAP. Design, Setting, and Participants This retrospective population-based cohort study was conducted among all patients with TOF born in the province of Quebec, Canada, from 1980 to 2015 who underwent complete surgical repair. Patients who received a TAP or VS procedure were matched using a propensity score based on preoperative factors in a 1:1 ratio. Data were analyzed from March 2020 through April 2021. Exposures The study groups were individuals who received TAP and those who received VS. The VS group was further stratified by the presence of residual pulmonary stenosis. Main Outcomes and Measures The primary outcome was all-cause mortality, with 30-year survival evaluated using Cox proportional-hazards models. Secondary outcomes included the cumulative mean number of cardiovascular interventions, pulmonary valve replacements (PVRs), and cardiovascular hospitalizations were evaluated using marginal means/rates regressions. Results Among 683 patients with TOF (401 patients who underwent TAP [58.7%] and 282 patients who underwent a VS procedure [41.3%]), adequate propensity score matching was achieved for 528 patients (264 patients who underwent a VS procedure and 264 patients who underwent TAP). Among this study cohort, 307 individuals (58.1%) were men. The median (interquartile range [IQR]) follow-up was 16.0 (8.1-25.4) years, for a total of 8881 patient-years, including 63 individuals (11.9%) followed up for more than 30 years. Individuals who received a VS procedure had an increased 30-year survival of 99.1% compared with 90.4% for individuals who received TAP (hazard ratio [HR], 0.09 [95% CI, 0.02-0.41]; P = .002). Patients who underwent TAP had an increased 30-year cumulative mean number of cardiovascular interventions compared with patients who underwent a VS procedure without residual pulmonary stenosis (2.0 interventions [95% CI, 1.5-2.7 interventions] vs 0.7 interventions [95% CI, 0.5-1.1 interventions]; mean ratio [MR], 0.36 [95% CI, 0.25-0.50]; P

In recent years, there has been a shift in the type of repair for tetralogy of Fallot (ToF) with a preference for valve-sparing (VS) surgery over transannular patching (TAP). However, long-term outcomes evaluation is lacking. The objective was to compare long-term outcomes of patients with ToF

Cardiac hemangioma is rare, even more when leading to a cardiovascular collapse in a seemingly healthy newborn. A 6-day-old neonate had a tamponade caused by a basolateral hemangioma of the left ventricle. Partial surgical resection was performed. A congenital lobular capillary hemangioma was diagnosed upon histologic examination. The patient recovered completely and shows normal development at the 12-month follow-up.

During the fall, a 17-day-old boy was brought to the emergency department because of progressive feeding difficulty and lethargy after developing nasal congestion three days before presentation. The infant was born following an uncomplicated full term pregnancy and delivery. The family history was notable only for a five-year-old brother with a bicuspid aortic valve. Both his parents and brother had been recently ill with a cold. The infant was hypotonic and pale with signs of respiratory distress. His heart rate was 140 beats/min, respiratory rate was 64 breaths/min, systolic blood pressure was approximately 80 mmHg, oxygen saturation was 90% on room air and his rectal temperature was 36.5°C. Physical examination revealed hepatomegaly (4 cm to 5 cm below the costal margin) and diminished femoral and radial pulses; no rash was noted. Fluid resuscitation was begun immediately. However, after receiving 60 mL/kg of crystalloid solution, the patient became bradycardic (60 beats/min), requiring chest compressions, intubation and continuous infusion of vasopressors for stabilization. Initial laboratory findings included a mixed respiratory and metabolic acidosis, lymphocytosis, elevated transaminases and evidence of early disseminated intravascular coagulopathy. A chest x-ray demonstrated cardiomegaly (Figure 1). Blood, cerebrospinal fluid (CSF) and urine samples were sent for bacterial culture. Broad-spectrum antibiotics were begun, and the patient was admitted to the paediatric intensive care unit. Further investigations revealed the diagnosis. Figure 1) Chest radiographs at presentation. Anteroposterior (A) and lateral (B) views demonstrate cardiomegaly and mildly increased vascular markings. There is no evidence of parenchymal pulmonary disease.

Objective Aortopulmonary collaterals are a frequent phenomenon in patients after bidirectional cavopulmonary connection. The aortopulmonary collateral flow volume can be quantified using cardiac magnetic resonance imaging. However, the significance of aortopulmonary collateral flow for the postoperative outcome after total cavopulmonary connection is unclear and was sought to be determined. Methods The data from 33 patients were prospectively studied with cardiac magnetic resonance, echocardiography, and cardiac catheterization before the total cavopulmonary connection operation. The early postoperative outcomes after total cavopulmonary connection completion were recorded. Results Aortopulmonary collateral flow was 1.59 L/min/m 2 ± 0.65 L/min/m 2 (range, 0.54 L/min/m 2 –3.34 L/min/m 2 ), constituting 43% ± 13% (range, 12–87%) of pulmonary blood flow and 35% ± 12% (range, 11–62%) of the cardiac index, resulting in a pulmonary blood flow/systemic blood flow ratio of 1.06 ± 0.17 (range, 0.79–1.55). The aortopulmonary collateral flow correlated with pulmonary blood flow/systemic blood flow ratio ( r = 0.69, P r = 0.42, P = .018), and cardiac index ( r = 0.53, P = .002). Of the 36 patients, 24 underwent fenestrated total cavopulmonary connection during the study period. The aortopulmonary collateral flow, relative to the cardiac index, correlated with the duration of hospital stay ( r = 0.48, P = .02) and pleural drainage ( r = 0.45, P = .03). Patients whose pleural drainage lasted 1 week or less had less aortopulmonary collateral flow before the Fontan operation than those with a longer period until chest tube removal (1.23 L/min/m 2 ± 0.38 L/min/m 2 vs 1.73 L/min/m 2 ± 0.76 L/min/m 2 ; P = .03). Compared with a contemporary group of total cavopulmonary connection patients with fenestration in their extracardiac conduit who were studied prospectively, with a similar protocol, the bidirectional cavopulmonary connection had a greater amount of aortopulmonary collateral flow (1.59 L/min/m 2 ± 0.65 L/min/m 2 vs 1.30 L/min/m 2 ± 0.57 L/min/m 2 , P = .04). Conclusions Patients after bidirectional cavopulmonary connection routinely acquire a large amount of aortopulmonary collateral flow. The hemodynamic consequences of aortopulmonary collateral flow translate into adverse outcomes early after total cavopulmonary connection completion.

Background The management of pediatric discrete subaortic stenosis remains controversial. Objectives Document the natural history and surgical outcomes for discrete subaortic stenosis to adolescence. Methods Retrospective review of clinical and echocardiographic findings in 74 patients diagnosed in childhood between 1985 and 1998. Results Twenty-five patients were followed only medically for 9.4 ± 0.9 years to 15.9 ± 0.6 years of age. Their echocardiographic left ventricular outflow peak gradient did not progress, 19 ± 1.4 (SEM) vs 20 ± 2.3 mm Hg. The proportion with aortic insufficiency (AI) increased (4% to 52%). Forty-nine patients were operated for discrete subaortic stenosis at 7.8 ± 0.6 years. Their peak gradient at diagnosis was 36 ± 3 mm Hg with AI in 33%. Preoperatively their peak gradient progressed to 60 ± 5 mm Hg with AI in 82%. Assessment 6.2 ± 0.5 years postoperativly showed a peak gradient of 14 ± 2 mm Hg with AI in 88%. Ten patients required reoperation for recurrent discrete subaortic stenosis, 3 acquired complete heart block, and 1 developed endocarditis. There was no mortality. At diagnosis, surgical patients were younger, had greater peak gradients, and greater incidence of AI, than those followed only medically. The progression of discrete subaortic stenosis was positively associated with severity of obstruction and negatively associated with older age at diagnosis. The risk of having surgery over time was associated with greater preoperative obstruction and presence of AI. Conclusions Many pediatric patients with mild discrete subaortic stenosis exhibit little progression of obstruction and need not undergo immediate surgery. Others with more severe stenosis may progress precipitously and will benefit from early resection.