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Tricuspid atresia with absent pulmonary valve: A rare form of single ventricle

Naik, Ronak; Makadia, Luv D; Ramirez, Michelle; Crawford, Maya T; Ahmad, Latifah; Kumar, T K Susheel
Tricuspid atresia with an absent pulmonary valve is a rare congenital cardiac defect. Although extensive pathological reviews have been published in the past, there are only a handful of cases that have been successfully palliated to the stage of Fontan. We hereby describe the successful management of one such case and review the surgical strategies described in the literature.
PMID: 35434814
ISSN: 1540-8191
CID: 5232782

Aortic migration of Amplatzer Piccoloâ„¢ ductal Occluder

Minocha, Prashant K; Sutton, Nicole; Crawford, Maya T; Williams, David; Ramirez, Michelle; Chakravarti, Sujata; Mosca, Ralph; Kumar, T K Susheel
We present the case of a 4-month-old, former 23-week premature baby who underwent patent ductus arteriosus device closure in the cardiac catheterisation lab with an Amplatzer Piccoloâ„¢ device at 12 weeks of life. This was complicated by late migration of the device into the aorta resulting in severe obstruction and requiring surgical intervention.
PMID: 35199639
ISSN: 1467-1107
CID: 5172272

Aortic migration of Amplatzer Piccolo (TM) ductal Occluder

Minocha, Prashant K.; Sutton, Nicole; Crawford, Maya T.; Williams, David; Ramirez, Michelle; Chakravarti, Sujata; Mosca, Ralph; Kumar, T. K. Susheel
ISI:000760926200001
ISSN: 1047-9511
CID: 5243012

Surgical Management of Giant Intrapericardial Teratoma Encasing the Coronary Artery

Minocha, Prashant; Hodzic, Emina; Sharma, Madhu; Bhatla, Puneet; Nielsen, James; Ramirez, Michelle; Magid, Margret; Fisher, Jason C; Mosca, Ralph; Kumar, Tk Susheel
Intrapericardial teratomas are rare, predominantly benign tumors that warrant surgical resection in the neonatal period because of their potential detrimental effects on the cardiorespiratory system. Surgical resection can be a challenge when the tumor encases and obscures a coronary artery. Adherence to certain operative principles is necessary to achieve successful outcomes.
PMID: 33888026
ISSN: 2150-136x
CID: 4852092

Cardiac involvement in a pediatric patient with COVID-19: Looking beyond the nonspecific global cardiac injury

Bhansali, Suneet; Minocha, Prashant; Phoon, Colin; Henry, Gillian; Chakravarti, Sujata; Ramirez, Michelle; Bhatla, Puneet
We report a case of a 17-year-old healthy male presenting with multisystem hyperinflammatory shock temporally associated with COVID-19. Cardiac involvement was suspected based on evidence of significant cardiac injury (elevated cardiac biomarkers, electrocardiographic and echocardiographic abnormalities). Cardiac magnetic resonance imaging was performed demonstrating global biventricular systolic dysfunction, as well as a small area of T2 hyperintensity and mid-wall late gadolinium enhancement. This case discusses the varied cardiac involvement in pediatric patients with COVID-19 infection and highlights that cardiac injury is not just limited to hyperinflammatory syndrome related global dysfunction but a more focal myocarditis can also be seen.
PMID: 32770794
ISSN: 1540-8175
CID: 4560682

Safety Checklist Implementation Did Not Reduce Central Venous Catheter Duration in Pediatric Cardiac ICU Patients

Sahulee, Raj; Ramirez, Michelle M; Al-Qaqaa, Yasir M; Chakravarti, Sujata B; McKinstry, Jaclyn
The Center for Disease Control recommends prompt removal of nonessential central venous catheters (CVCs) to reduce the risk for central line-associated bloodstream infections. Safety checklists have been trialed to reduce nonessential CVC days, but pediatric studies are lacking. Our specific aim was to detect >10% reduction in mean CVC duration after implementation of a safety checklist addressing CVCs in our unit.
PMCID:7056290
PMID: 32190798
ISSN: 2472-0054
CID: 4352882

Successful Phenobarbital Desensitization After DRESS Reaction in the Management of Refractory Status Epilepticus

Witcher, Robert H; Ramirez, Michelle M
PURPOSE/OBJECTIVE:Drug reaction with eosinophilia and systemic symptoms (DRESS) is associated with antiepileptic drug use and is a rare but life-threatening side effect. We present a case of phenobarbital-induced DRESS in a patient who subsequently required phenobarbital and was successfully desensitized. SUMMARY/CONCLUSIONS:A 5-year-old male presented with medically refractory status epilepticus (SE). He had been trialed on several antiepileptic medications without achieving burst suppression. Burst suppression was achieved with a pentobarbital infusion, and thus, phenobarbital was initiated as the pentobarbital was weaned. After five days of phenobarbital, the patient developed signs and symptoms concerning for DRESS; a punch biopsy confirmed the drug reaction. Two months later, he again developed SE unresponsive to antiepileptic infusions. Burst suppression was achieved with pentobarbital, and it was decided to transition the patient to phenobarbital. Due to concerns of phenobarbital-induced DRESS, the patient underwent a phenobarbital desensitization consisting of 6 doses sequentially administered in 10-fold increasing concentrations before achieving therapeutic dosing. Three days later, the patient achieved therapeutic phenobarbital levels, was weaned off of pentobarbital, and remained seizure-free without recurrence of DRESS. CONCLUSIONS:Graded desensitization may be an option to minimize recurrence of DRESS in patients where avoidance of the offending agent is not possible.
PMID: 29390946
ISSN: 1531-1937
CID: 2933902

MALIGNANT HYPERTHERMIA-ASSOCIATED LIVER FAILURE TREATED WITH N-ACETYLCYSTEINE IN A 5-YEAR-OLD BOY [Meeting Abstract]

Dapul, Heda; Chopra, Arun; Cohn, Moshe; Ramirez, Michelle; Santos, Laura; Wen, Andy; Zawistowski, Christine; Al-Qaqaa, Yasir
ISI:000436794300493
ISSN: 0090-3493
CID: 3507712

Novel Use of an Ultrafiltration Device as an Alternative Method for Fluid Removal in Critically Ill Pediatric Patients with Cardiac Disease: A Case Series

Chakravarti, Sujata; Al-Qaqaa, Yasir; Faulkner, Meghan; Bhatla, Puneet; Argilla, Michael; Ramirez, Michelle
Fluid overload (FO) is a common complication for pediatric patients in the intensive care unit. When conventional therapy fails, hemodialysis or peritoneal dialysis is classically used for fluid removal. Unfortunately, these therapies are often associated with cardiovascular or respiratory instability. Ultrafiltration, using devices such as the Aquadex system (Baxter Healthcare, Deerfield, IL, USA), is an effective tool for fluid removal in adult patients with congestive heart failure. As compared to hemodialysis, ultrafiltration can be performed using smaller catheters, and the extracorporeal volume and minimal blood flow rates are lower. In addition, there is no associated abdominal distension as is seen in peritoneal dialysis. Consequently, ultrafiltration may be better tolerated in critically ill pediatric patients. We present three cases of challenging pediatric patients with FO in the setting of congenital heart disease in whom ultrafiltration using the Aquadex system was successfully utilized for fluid removal while cardiorespiratory stability was maintained.
PMCID:4933814
PMID: 27433308
ISSN: 2036-749x
CID: 2184952

Multiple organ dysfunction syndrome

Ramirez, Michelle
Initially known as multiple system organ failure, the term multiple organ dysfunction syndrome (MODS) was first described in the 1960s in adults with bleeding, respiratory failure, and sepsis. It is defined as "the development of potentially reversible physiologic derangement involving two or more organ systems not involved in the disorder that resulted in ICU admission, and arising in the wake of a potentially life threatening physiologic insult."(3) There are many risk factors predisposing to MODS; however, the most common risk factors are shock due to any cause, sepsis, and tissue hypoperfusion. A dysregulated immune response, or immuneparalysis, in which the homeostasis between pro-inflammatory and anti-inflammatory reaction is lost is thought to be key in the development of MODS. The clinical course and evolution of MODS is dependent on a combination of acquired and genetic factors. There are several nonspecific therapies for the prevention and resolution of MODS, mostly care is supportive. Mortality from MODS in septic pediatric patients varies between 11% and 54%.
PMID: 24295608
ISSN: 1538-3199
CID: 666392