A 19-Year-Old Pregnant Woman With Pulmonary Hypertension With Progressive Dyspnea.

CASE PRESENTATION: A 19-year-old pregnant woman at week 32 of gestation was referred to our clinic with progressive shortness of breath for the further evaluation and treatment of high-risk pregnancy. Her complaints had been existing since her childhood. Two years prior to her admission, she had been diagnosed with heart failure with preserved ejection fraction due to cardiomyopathy and associated pulmonary hypertension. The patient had no family history of any cardiac disease. She had never smoked or drunk alcohol. Her clinical condition had deteriorated progressively with the pregnancy.

Tratamiento anticoagulante y vasodilatador para el síndrome de Nicolau tras la administración de una inyección de bencilpenicilina benzatínica intramuscular a un niño de cuatro años de edad / Anticoagulant and vasodilator therapy for Nicolau syndrome following intramuscular benzathine penicillin injection in a 4 year old boy

El síndrome de Nicolau (SN) es una complicación rara de la inyección de ciertos fármacos por vía intramuscular, intrarticular o subcutánea, que produce necrosis isquémica de la piel, las partes blandas y el tejido muscular circundante. La bencilpenicilina benzatínica es uno de los antibióticos más ampliamente empleados para las infecciones de las vías respiratorias altas y raramente se ha notificado que produzca SN. En este artículo presentamos el caso de un niño de sexo masculino de cuatro años de edad diagnosticado con SN tras la inyección de bencilpenicilina benzatínica tratado satisfactoriamente con heparina fraccionada (enoxaparina) y pentoxifilina. Los médicos deben estar atentos al uso innecesario de bencilpenicilina benzatínica para evitar las probables complicaciones.

Nicolau syndrome (NS) is a rare complication of intramuscular, intraarticular or subcutaneous injection of particular drugs leading to ischemic necrosis of the surrounding skin, soft tissue and muscular tissue. Benzathine penicilin one of the most widely used antibiotic for upper respiratory tract infections and has been rarely reported to cause NS. Here we describe a 4 year old boy with diagnosis of NS after the injection of benzathine penicillin who was successfuly treated with unfractionized heparin (enoxaparine) and pentoxifylline. The practitioners should pay attention for unnecessary use of benzathine penicillin to avoid from probable complications.

Anticoagulant and vasodilator therapy for Nicolau syndrome following intramuscular benzathine penicillin injection in a 4 year old boy. / Tratamiento anticoagulante y vasodilatador para el síndrome de Nicolau tras la administración de una inyección de bencilpenicilina benzatínica intramuscular a un niño de cuatro años de edad.

Nicolau syndrome (NS) is a rare complication of intramuscular, intraarticular or subcutaneous injection of particular drugs leading to ischemic necrosis of the surrounding skin, soft tissue and muscular tissue. Benzathine penicilin one of the most widely used antibiotic for upper respiratory tract infections and has been rarely reported to cause NS. Here we describe a 4 year old boy with diagnosis of NS after the injection of benzathine penicillin who was successfuly treated with unfractionized heparin (enoxaparine) and pentoxifylline. The practitioners should pay attention for unnecessary use of benzathine penicillin to avoid from probable complications.

El síndrome de Nicolau (SN) es una complicación rara de la inyección de ciertos fármacos por vía intramuscular, intrarticular o subcutánea, que produce necrosis isquémica de la piel, las partes blandas y el tejido muscular circundante. La bencilpenicilina benzatínica es uno de los antibióticos más ampliamente empleados para las infecciones de las vías respiratorias altas y raramente se ha notificado que produzca SN. En este artículo presentamos el caso de un niño de sexo masculino de cuatro años de edad diagnosticado con SN tras la inyección de bencilpenicilina benzatínica tratado satisfactoriamente con heparina fraccionada (enoxaparina) y pentoxifilina. Los médicos deben estar atentos al uso innecesario de bencilpenicilina benzatínica para evitar las probables complicaciones.

Are perioperative near-infrared spectroscopy values correlated with clinical and biochemical parameters in cyanotic and acyanotic infants following corrective cardiac surgery?

BACKGROUND: Near-infrared spectroscopy (NIRS) is a useful non-invasive tool for monitoring infants undergoing cardiac surgery. In this study, we aimed to determine the NIRS values in cyanotic and acyanotic patients who underwent corrective cardiac surgery for congenital heart diseases. METHODS: Thirty consecutive infants who were operated on with the diagnosis of ventricular septal defect (n=15) and tetralogy of Fallot (n=15) were evaluated retrospectively. A definitive repair of the underlying cardiac pathology was achieved in all cases. A total of six measurements of cerebral and renal NIRS were performed at different stages of the perioperative period. The laboratory data, mean urine output and serum lactate levels were evaluated along with NIRS values in each group. RESULTS: The NIRS values differ in both groups, even after the corrective surgical procedure is performed. The recovery of renal NIRS values is delayed in the cyanotic patients. CONCLUSION: Even though definitive surgical repair is performed in cyanotic infants, recovery of the renal vasculature may be delayed by up to two days, which is suggestive of a vulnerable period for renal dysfunction.

Correlation between cerebral-renal near-infrared spectroscopy and ipsilateral renal perfusion parameters as clinical outcome predictors after open heart surgery in neonates and infants.

The objective of this clinical study is to determine whether correlation exists among cerebral and renal near-infrared spectroscopy (NIRS) measurements, renal Doppler ultrasonography parameters (resistive index, peak systolic velocity), and early postoperative clinical outcomes following cardiac surgery in neonates and infants. Thirty-seven patients undergoing surgery for congenital heart defects with an age of less than 3 months, all of whom were in the high-risk group according to Aristotle Basic Complexity risk stratification score, were enrolled in our study. Cerebral, renal NIRS values and renal Doppler ultrasonography measurements were recorded for each patient at the 4th postoperative hour. The renal resistive indices were calculated for each case, and the patients were divided into two groups according to renal resistive index (RI) values. Group I included the patients with a RI of greater than 0.8 (n = 25) and Group II included the patients with a RI of less than 0.8 (n = 12). The postoperative outcome parameters were compared in between two groups. Group I (RI >0.8) had lower postoperative mean urine output than Group II (RI <0.8) (P = 0.041). The lactate levels were significantly higher in Group I (P = 0.049), as well. The postoperative intensive care unit and hospital stay of Group I was significantly higher than Group II (P = 0.048). Both cerebral and renal NIRS values and the assessment of renal RI as well as peak systolic values can be used in order to predict the early clinical outcome in cardiac surgery patients in early infantile and neonatal period.

Impact of pulsatile perfusion on clinical outcomes of neonates and infants with complex pathologies undergoing cardiopulmonary bypass procedures.

The aim of this clinical trial was to evaluate the pulsatile perfusion mode in pediatric patients who had complex cardiac pathologies according to Jenkins stratifications (category 4) undergoing cardiopulmonary bypass procedures (CPB). Patients with transposition of great arteries (TGA) and ventricular septal defect (VSD) were included in this clinical study. Eighty-nine consecutive pediatric patients undergoing open heart surgery for repair of TGA-VSD were prospectively entered into the study and were randomly assigned to either the pulsatile perfusion group (Group P, n = 58) or the nonpulsatile perfusion group (Group NP, n = 31). There were no differences between groups in terms of demographical and intraoperative parameters. The pulsatile group needed significantly less inotropic support (P < 0.05) and had lower lactate levels (P < 0.001), higher urine output (P < 0.01), and higher albumin levels (P < 0.05). In addition, the pulsatile group had less ICU (P < 0.01) and hospital stays (P < 0.001). We conclude that the use of pulsatile flow is a better option and should be considered for repair of the complex congenital heart defects.

Translational research in pediatric extracorporeal life support systems and cardiopulmonary bypass procedures: 2011 update.

Over the past 6 years at Penn State Hershey, we have established the pediatric cardiovascular research center with a multidisciplinary research team with the goal to improve the outcomes for children undergoing cardiac surgery with cardiopulmonary bypass (CPB) and extracorporeal life support (ECLS). Due to the variety of commercially available pediatric CPB and ECLS devices, both in vitro and in vivo translational research have been conducted to achieve the optimal choice for our patients. By now, every component being used in our clinical settings in Penn State Hershey has been selected based on the results of our translational research. The objective of this review is to summarize our translational research in Penn State Hershey Pediatric Cardiovascular Research Center and to share the latest results with all the interested centers.

Evaluation of perfusion modes on vital organ recovery and thyroid hormone homeostasis in pediatric patients undergoing cardiopulmonary bypass.

The objectives of this study were: (i) to evaluate the effects of perfusion modes (pulsatile vs. nonpulsatile) on vital organs recovery and (ii) to investigate the influences of two different perfusion modes on the homeostasis of thyroid hormones in pediatric patients undergoing cardiopulmonary bypass (CPB) procedures. Two hundred and eighty-nine consecutive pediatric patients undergoing open heart surgery for repair of congenital heart disease were prospectively entered into the study and were randomly assigned to two groups: the pulsatile perfusion group (Group P, n = 208) and the nonpulsatile perfusion group (Group NP, n = 81). All patients received identical surgical, perfusional, and postoperative care. Study parameters included total drainage, mean urine output in the intensive care unit (ICU), intubation time, duration of ICU and hospital stay, the need for inotropic support, pre- and postoperative enzyme levels (ALT [alanine aminotransaminase] and AST [aspartate aminotransaminase]), c-reactive protein, lactate, albumin, blood count (leukocytes, hematocrit, platelets), creatinine levels, and thyroid hormones (thyroid stimulating hormone [TSH], FT(3) [free triiodothyronine], FT(4) [free thyroxine]). All patients survived the perioperative and postoperative periods. There were no statistically significant differences in either preoperative or operative parameters between the two groups. Group P, compared to Group NP, required significantly less inotropic support, had a shorter intubation period, higher urine output in ICU, and shorter duration of ICU and hospital stay. Lower lactate levels and higher albumin levels were observed in Group P and there were no significant differences in creatinine, enzyme levels, blood counts, or drainage amounts between two groups. TSH, Total T(3) , Total T(4) , and FT(3) , FT(4) levels were markedly reduced versus their preoperative values in both groups. FT(3) and FT(4) levels were reduced significantly further in the nonpulsatile group both during CPB and at 72 h postoperation. The results of this study confirm our opinion that pulsatile perfusion leads to better vital organ recovery and clinical outcomes in the early postoperative period as compared to nonpulsatile perfusion in pediatric patients undergoing CPB cardiac surgery. The plasma concentrations of thyroid hormones are dramatically reduced during and after CPB, but pulsatile perfusion seems to have a protective effect of thyroid hormone homeostasis compared to nonpulsatile perfusion.

Cardiac surgery of premature and low birthweight newborns: is a change of fate possible?

Low birthweight (LBW) continues to be a high-risk factor in surgery for congenital heart disease. This risk is particularly very high in very low birthweight infants under 1500g and extremely LBW infants under 1000g. From January 2005 to December 2008, 33 consecutive LBW neonates underwent cardiac surgery in our clinic in keeping with the criteria for choice of surgery. Their weight range was between 800 and 1900g. Nine of them were under 1000g. Cardiopulmonary bypass (CPB) was used in 17 patients (39.5%) and pulsatile perfusion mode was applied to patients in the CPB group. The same surgical team operated to achieve palliation (8 patients, 24.2%) or full repair (25 patients, 75.8%). Median gestational age was 36 weeks with 12 (36.4%) premature babies (≤37 weeks). Median age at operation was 5 days. Pathologies were single ventricle (n=3), pulmonary atresia-ventricular septal defect (n=3), aortic coarctation (n=10), aorticopulmonary window and interrupted aortic arch combination (n=6), patent arterial duct (n=11), critical aortic stenosis (n=8), and tetralogy of Fallot with pulmonary atresia (n=2). One infant had VATER syndrome. Selective cerebral perfusion technique was used in complex arch pathologies for cerebral protection. Median follow-up was 14 months. There were four early postoperative deaths. None of the cases showed a need for early reoperation. The acceptable early- and midterm mortality rates in this group suggest that these operations can be successfully performed. There is a need for further multicenter studies to evaluate these high-risk groups.