Short term outcome of neonatal venous thromboembolism in anticoagulated versus observed patients.

BACKGROUND: Advancements in the medical field and increased survival of premature infants have led to a rise of venous thromboembolism (VTE) in neonates. Neonatal hemostasis exists in a delicate balance with a propensity towards pro-coagulation. Current recommendations include careful observation, therapeutic anti-coagulation and in some cases thrombolysis. We hypothesize that a sub-group of neonates may not require anti-coagulation and could be safely observed thus reducing their risk of bleeding complications. METHODS: We conducted a retrospective chart review of patients with VTE admitted to the neonatal intensive care unit at the Oklahoma University Children's Hospital from October 2009-October 2019. Patients were selected if they had an ICD-9 or ICD-10 code specific for a VTE or if screening with CPT codes for diagnostic imaging including echocardiogram, venous dopplers and computed topographic/magnetic resonance venogram revealed the word 'thrombus', 'clot' or 'venous thromboembolism'. Data were collected about demographics, medical history, co-morbidities, thrombosis characteristics, treatment and outcome. RESULTS: A total of 211 patients were screened and 119 patients were eligible and included in the study. The majority of patients (85 %) had a central venous catheter (CVC) associated VTE. Two-thirds of patients (n = 81, 68 %) received therapeutic anti-coagulation while one-third (n = 38, 32 %) were observed. The group that received anticoagulation had a significantly older age at diagnosis and had a higher frequency of bacteremia, congenital heart disease and presence of symptoms. There was no difference in the odds of complete resolution between patients who were treated with therapeutic anti-coagulation and those that were observed (OR: 1.37, 95 % CI: 0.59-3.20, p-value: 0.47). Univariate analysis revealed maternal preeclampsia (OR: 0.2, 95 % CI: 0.05-0.82, p-value = 0.025), maternal history of chronic hypertension (OR: 0.17, 95 % CI: 0.04-0.68, p-value = 0.01), and presence of complete occlusion (OR = 0.37, 95 % CI: 0.15-0.91, p-value = 0.03) significantly reduced the odds of complete resolution. Furthermore, having a VTE related to a CVC in an extremity versus an ECMO cannula or cardiac catheterization significantly improved the odds of VTE resolution (OR = 5.94, 95 % CI: 1.30-27.20, p-value = 0.022). Using a stepwise regression model, maternal history of chronic hypertension remained significant for a reduced odds of VTE resolution (OR: 0.14, 95 % CI 0.025-0.73, p-value: 0.02). CONCLUSIONS: The short-term outcome of neonatal VTE does not seem to differ between those that were anticoagulated and those that were observed with serial imaging.

Hereditary Thrombotic Thrombocytopenic Purpura.

Hereditary thrombotic thrombocytopenic purpura (hTTP), also known as Upshaw-Schulman syndrome, is a rare genetic disorder caused by mutations in the ADAMTS13 gene that leads to decreased or absent production of the plasma von Willebrand factor (VWF)-cleaving metalloprotease ADAMTS13. The result is circulating ultra-large multimers of VWF that can cause microthrombi, intravascular occlusion and organ damage, especially at times of turbulent circulation. Patients with hTTP may have many overt or clinically silent manifestations, and a high index of suspicion is required for diagnosis. For the treatment of hTTP, the goal is simply replacement of ADAMTS13. The primary treatment is prophylaxis with plasma infusions or plasma-derived factor VIII products, providing sufficient ADAMTS13 to prevent acute episodes. When acute episodes occur, prophylaxis is intensified. Recombinant ADAMTS13, which is near to approval, will immediately be the most effective and also the most convenient treatment. In this review, we discuss the possible clinical manifestations of this rare disease and the relevant differential diagnoses in different age groups. An extensive discussion on prophylaxis and treatment strategies is also presented. Unique real patient cases have been added to highlight critical aspects of hTTP manifestations, diagnosis and treatment.

Venous Thromboembolism in Premature Neonates.

While the incidence of venous thromboembolism (VTE) is lower among children than adults, the newborn period is one of two bimodal peaks (along with adolescence) in VTE incidence in the pediatric population. Most VTE cases in neonates occur among critically ill neonates being managed in the neonatal intensive care unit, and most of these children are born premature. For this reason, the presentation, diagnosis, management, and outcomes of VTE among children born premature deserve special emphasis by pediatric hematologists, neonatologists, pharmacists, and other pediatric health care providers, as well as by the scientific community, and are described in this review.

Aorta-to-left atrial fistula developing after surgical removal of an atrial septal occlusion device eight years after original implantation.

Cardiac erosions may occur in a small percentage of patients after device closure of atrial septal defects. These devices have to be explanted. We report an aorta-to-left atrial fistula after surgical explantation of the device eight years after implant for access to mitral valve repair. The importance of realizing the risk of subclinical cardiac erosion and subsequent fistula development after device removal is discussed.

Distinctive Hemodynamics in the Immediate Postoperative Period of Patients with a Longer Cardiac Intensive Care Stay Post-Tetralogy of Fallot Repair.

OBJECTIVE: After complete surgical repair the majority of tetralogy of Fallot (ToF), patients stay ≤2 days in the Cardiac Intensive Care Unit (CICU) while some may stay longer. We undertook this study to investigate the factors associated with shorter vs. longer length of stay in the CICU to help manage resources effectively. DESIGN: Patients who underwent ToF repair at Aga Khan University, Pakistan, between July 2006 and December 2011 were studied in a case-control design. Clinical parameters were compared between short stay group (SSG) (≤2 days) and long stay group (LSG) (>2 days). Odds ratios were calculated, and regression was performed. RESULTS: Ninety-eight patients (LSG 65, SSG 33) were included. Patients with lower preoperative saturation were 2.67 times more likely to be in the LSG group (P = .02). At 4 hours postoperatively, patients with a higher inotropic score (odds ratio [OR] = 3.03, confidence interval [CI] = 1.19-7.7, P = .02), higher central venous pressure (OR = 3.04, CI = 1.27-7.32, P = .013), and significant tachycardia at 4 hours (OR = 3.5, CI = 1.19-10.3. P = .02) were at risk for having a prolonged CICU stay. On multivariate analysis, significant postoperative tachycardia at 4 hours (z-score ≥3) was highly specific (sensitivity = 38.5%, specificity = 84.9%) for predicting the chances of being in the LSG. Other predictors included preop O(2) saturation ≤82.5% (sensitivity = 61.1%, specificity = 63.0%) and CVP ≥10 mm Hg at 4 hours (sensitivity = 55.4%, specificity = 71.9%). CONCLUSION: Patients who end up staying longer in the CICU have features that are distinctive in the immediate postoperative period, and this can help clinicians in identifying patients who may need more support.