Pentalogy of Fallot (PoF) is a congenital heart disease comprising tetralogy of Fallot plus an atrial septal defect (ASD). Patients are diagnosed early in life and submitted to reparative surgery. Without it, the prognosis is poor. This female patient, initially diagnosed with transposition of great arteries (TGA), ASD and a ventricular septal defect, got pregnant at 26 years old and had an early delivery due to fetal distress. She resumed follow-up, and her last echocardiogram put in doubt the diagnosis of TGA. Cardiac CT then revealed a PoF as well as pulmonary arteriovenous fistulas and a persistent left superior vena cava.

BACKGROUND: DOORS syndrome (deafness, onychodystrophy, osteodystrophy, mental retardation and seizures; MIM 220500) is a rare multisystem genetic disorder, mainly characterized by sensorineural deafness, shortened terminal phalanges with small nails of hands and feet, intellectual deficit, and seizures. The disease is caused by homozygous or compound heterozygous mutation in the TBC1 domain family member 24 (TBC1D24) gene (gene locus/MIM 613577) on chromosome 16p13.
OBJECTIVE: We report the first case of DOORS syndrome from Indonesia.
METHODS: A review of the literature was conducted and cases compared.
RESULTS: A 27-day-old baby girl was brought to us with a history of recurrent seizures and absence of all finger- and toenails since birth. In addition, physical examination revealed left eye strabismus and a single transverse palmar crease on both hands. X-rays of the hands and feet showed absence of the distal phalanx of her right and left fingers II-V and the distal phalanx of her right and left toes I-V, respectively. Brainstem-evoked response audiometry test revealed profound bilateral sensorineural deafness. Pentalogy of Fallot was diagnosed by echocardiography, while an abnormal diffuse epileptiform pattern was found on electroencephalography.
CONCLUSIONS: This is the first report of an association between pentalogy of Fallot and single transverse palmar crease in DOORS syndrome.

Pentalogy of Fallot is a rare congenital cyanotic heart disease and a variant of tetralogy of Fallot, in which tetralogy of Fallot is associated additionally with an atrial septal defect. It is characterized by right to left intracardiac shunting of blood leading to decreased pulmonary blood flow resulting in the development of cyanotic episodes. The case report aims to present the dental management of an 8-year-old child with a repaired cardiac anomaly of pentalogy of Fallot. His medical condition was assessed and physician consent was sought for procedural intervention. All invasive procedures were planned under antibiotic prophylaxis to prevent bacteremia associated endocarditis. Elaborate chairside dental intervention was carried out beginning with preventive schedule, oral prophylaxis and topical fluoride application followed by interception of carious progression with multiple GIC restorations and extractions of retained and grossly decayed teeth. Patient was reviewed on follow-up visit and no pain, discomfort, or complications were reported. Based on the case report it can be concluded that the medical status in patients affected with Pentalogy of Fallot must be taken into consideration to plan dental treatment and precautions must be taken for prevention of stress induced cyanosis and bacterial endocarditis in such cases.

An 18-month-old female Holstein Friesian heifer presented with a history of stunted growth and a recent onset of anorexia; she presented with cyanosis and eventually died. A postmortem examination revealed obstruction of the right ventricular outflow tract, ventricular septal defect, overriding aortic root, right ventricular hypertrophy, and an atrial septal defect, indicating a pentalogy of Fallot (POF). In addition to POF, the heifer also had pulmonary artery dilatation, although she did not present with patent ductus arteriosus. This heifer had the longest lifespan among the Holstein cattle reported to have POF, which may be secondary to delayed pulmonary obstruction due to deformation of one of the pulmonary valves.

We present a 31-year-old primigravida with uncorrected pentalogy of Fallot, pregnant with monochorionic-diamniotic twins, undergoing elective lower segment cesarean section at 36 weeks gestation. Preoperative workup included a transthoracic echocardiogram which revealed a large ventricular septal defect of 1.8 cm with bidirectional shunting, a moderate size atrial septal defect of 1.8 cm with predominant left-to-right shunting, an overriding aorta, moderate right ventricular hypertrophy, and severe pulmonary valve stenosis. Notably, the patient was acyanotic with normal effort tolerance. Preoperative preparation involved the input of cardiologists and obstetric and cardiothoracic anesthetists. Issues such as the use of extracorporeal membrane oxygenation and cardiopulmonary support in the event of cardiac failure were discussed. Autotransfusion postdelivery was also addressed, and plans made for therapeutic venesection should need to arise. Intraoperatively, the planned anesthetic technique was slow and titrated combined spinal-epidural. However, a general anesthetic technique with rapid sequence induction was used in view of extreme patient anxiety. Intravenous induction was performed with ketamine and etomidate, followed by paralysis with succinylcholine. Anesthesia was maintained with desflurane on a mixture of air and oxygen. Phenylephrine infusion was titrated according to the patient\'s blood pressure and systemic vascular resistance. The uterotonic of choice was duratocin given as a slow bolus, followed by a 4-h infusion of oxytocin. The patient was put in a head-up position to prevent venous air embolism and to decrease autotransfusion to central circulation. Postoperatively, she was extubated and sent to the Intensive Care Unit for continuous monitoring with FloTrac®.