Noonan syndrome is a condition involving facial, cardiovascular and skeletal abnormalities that may pose problems to anesthesiologists during surgery. Propofol, which is used as an induction agent for noncardiac surgery, produces little or no change in the heart rate. Remifentanil decreases the sympathetic and somatic responses to noxious stimuli and can be given in high doses without negative inotropic effects. We report successful management of a patient with Noonan syndrome, hypertrophic cardiomyopathy, and atrial fibrillation, undergoing laparoscopic cholecystectomy using the the total intravenous anesthesia with propofol and remifentanil.
Anesthesia, Intravenous* ; Atrial Fibrillation ; Cardiomyopathy, Hypertrophic ; Cholecystectomy, Laparoscopic ; Heart Rate ; Humans ; Noonan Syndrome* ; Propofol

BACKGROUND: Patent ductus arteriosus (PDA) ligation is usually performed by congenital cardiac surgeons. However, due to the uneven distribution of congenital cardiac surgeons in South Korea, many institutions depend solely on adult cardiac surgeons for congenital cardiac diseases. We report the outcomes of PDA ligations performed by adult cardiac surgeons at our institution. METHODS: The electronic medical records of 852 neonates at Chung-Ang University Hospital, Seoul, South Korea from November 2010 to May 2014 were reviewed to identify patients with PDA. RESULTS: Of the 111 neonates with a diagnosis of PDA, 26 (23%) underwent PDA ligation. PDAs were ligated within 28 days of birth (mean, 14.5±7.8 days), and the mean gestational age of these patients was 30.3±4.6 weeks (range, 26 to 40 weeks) with a mean birth weight of 1,292.5±703.5 g (range, 480 to 3,020 g). No residual shunts through the PDA were found on postoperative echocardiography. There was 1 case of 30-day mortality (3.8%) due to pneumonia, and 6 cases of in-hospital mortality (23.1%) after 30 days, which is comparable to results from other centers with congenital cardiac surgery programs. CONCLUSION: Although our outcomes may not be generalizable to all hospital settings without a congenital cardiac surgery program, in select centers, PDA ligations can be performed safely by adult cardiac surgeons if no congenital cardiac surgery program is available.
Adult* ; Birth Weight ; Cardiac Surgical Procedures ; Diagnosis ; Ductus Arteriosus, Patent* ; Echocardiography ; Electronic Health Records ; Gestational Age ; Heart Defects, Congenital ; Heart Diseases ; Hospital Mortality ; Humans ; Infant, Newborn ; Korea ; Ligation* ; Mortality ; Parturition ; Pneumonia ; Seoul ; Surgeons* ; Thoracic Surgery

Familial amyloidotic polyneuropathy (FAP) is a rare hereditary amyloidosis that is characterized by slowly progressive peripheral polyneuropathy with other systemic involvement. More than 100 amyloidogenic transthyretin gene mutations have been reported, mainly in endemic areas of Portugal, Japan, and Sweden. We describe two brothers who exhibited progressive painful sensorimotor polyneuropathy with autonomic dysfunction. Gene analysis revealed a heterozygous Asp38Ala substitution in the transthyretin gene; this represents the first reported case of FAP in Korea.
Amyloidosis ; Amyloidosis, Familial ; Humans ; Japan ; Korea ; Polyneuropathies ; Portugal ; Prealbumin ; Siblings ; Sweden

Background: Pericardial fat (PF) is highly associated with cardiovascular disease but the effectiveness of surgical resection of PF is still unknown for myocardial mitochondrial structure and function in acute myocardial infarction (AMI) with obesity. The aim of this study was to demonstrate the difference in myocardial mitochondrial structure and function between obese AMI with additionally resected PF and those without resected PF. Methods: Obese rats with 12-week high fat diet (45 kcal% fat, n = 21) were randomly assigned into 3 groups: obese control, obese AMI and obese AMI with additionally resected PF. One week after developing AMI and additional resection of PF, echocardiogram, myocardial mitochondrial histomorphology, oxidative phosphorylation system (OXPHOS), anti-oxidative enzyme and sarcoplasmic reticulum Ca 2+ ATPase 2 (SERCA2) in the non-infarcted area were assessed between these groups. Results: There was significant improvement of systolic function in AMI with PF resection compared with the AMI group in the echocardiogram. Even though the electron microscopic morphology for the mitochondria seems to be similar between the AMI with PF resection and AMI groups, there was an improved expression of PGC-1α and responsive OXPHOS including NDUFB3, NDUFB5 and SDHB are associated with the ATP levels in the AMI with PF resection compared with those in the AMI group. In addition, the expression levels of antioxidant enzymes (MnSOD) and SERCA2 were improved in the AMI with PF resection compared with those in the AMI group. Conclusion Surgical resection of PF might ameliorate myocardial mitochondria dysfunction in obese AMI.

Paradoxical embolism after the Valsalva maneuver can cause ischemic stroke in patients with patent foramen ovale (PFO). A 27-year-old woman presented with abnormal movement of the right arm and aphasia. Brain imaging showed acute infarction in the left frontal lobe and segmental steno-occlusion in the left middle cerebral artery. A PFO study demonstrated air embolic signals in the middle cerebral artery. This case of embolic stroke occurred during sexual intercourse, probably related to the Valsalva maneuver, in a patient with PFO.
Adult ; Aphasia ; Arm ; Cerebral Infarction ; Coitus ; Dyskinesias ; Embolism, Paradoxical ; Female ; Foramen Ovale, Patent ; Frontal Lobe ; Humans ; Infarction ; Middle Cerebral Artery ; Neuroimaging ; Stroke ; Valsalva Maneuver