Anestesia para cirugía convencional en paciente con tetralogía de Fallot. Presentación de un caso / Anesthesia for conventional surgery in a patient with Tetralogy of Fallot. Case presentation

La tetralogía de Fallot es la forma más frecuente de cardiopatía congénita cianótica que se presenta en los neonatos. Los cuatro componentes de la enfermedad son: la alineación anormal de la comunicación interventricular, la obstrucción infundibular del ventrículo derecho, el cabalgamiento aórtico de la comunicación interventricular y la hipertrofia del ventrículo derecho. En este trabajo se trata un caso que se presentó para una cirugía convencional frecuente, la Histerectomía Abdominal Total debido a un mioma uterino, en una paciente que padecía de tetralogía de Fallot, con 43 años de edad, situación poco frecuente en la práctica diaria. El objetivo de este trabajo es exponer la experiencia del caso, que necesitó un manejo cuidadoso y que no aparece en la literatura básica. Habitualmente se aplica anestesia para niños con esta malformación para mejorar la calidad de vida o corregirla definitivamente, y porque precisamente solo el 2 % de los pacientes con esta enfermedad, que no han sido tratados quirúrgicamente, pueden arribar a la cuarta década de vida (AU).

The tetralogy of Fallot is the most frequent form of cyanotic congenital heart diseases presented in newborns. The disease’s four components are: abnormal alignment of intraventricular communication, right ventricle infundibular obstruction, aortic straddling of intraventricular communication, and right ventricle hypertrophy. The case presented is a case of a frequent conventional surgery, the total abdominal hysterectomy due to a uterine myoma, in a patient, aged 43 years, suffering for tetralogy of Fallot, a little frequent situation in the daily practice. The objective is exposing the experience of the case, demanding a careful management, which was not found in the main literature sources. Anesthesia is usually used in children with this malformation to improve their life quality or it is definitively corrected; only 2 % of the patients suffering this disease that have not been surgically treated are still alive in the fourth decade of life (AU).

Perioperative management of a patient with Dandy Walker malformation with tetralogy of Fallot undergoing total correction and fresh homologous pericardial pulmonary valve conduit implantation: Report of a rare case.

Perioperative management of a patient with Dandy–Walker malformation (DWM) with tetralogy of Fallot (TOF), patent ductus arteriosus, and pulmonary artery stenosis is a great challenge to the anesthesiologist. Anesthetic management in such patients can trigger tet spells that might rapidly increase intracranial pressure (ICP), conning and even death. The increase in ICP can precipitate tet spells and further brain hypoxia. To avoid an increase in ICP during TOF corrective surgery ventriculo‑peritoneal (VP) shunt should be performed before cardiac surgery. We present the first case report of a 11‑month‑old male baby afflicted with DWM and TOF who underwent successful TOF total corrective surgery and fresh autologous pericardial pulmonary valve conduit implantation under cardiopulmonary bypass after 1 week of VP shunt insertion.

Effect of dexmeditomidine on postoperative junctional ectopic tachycardia after complete surgical repair of tetralogy of Fallot: A prospective randomized controlled study.

Introduction: Incidence of junctional ectopic tachycardia (JET) after repair of tetralogy of Fallot (TOF) is 5.6–14%. Dexmeditomidine is a α-2 adrenoceptor agonist modulates the release of catecholamine, resulting in bradycardia and hypotension. These effects are being explored as a therapeutic option for the prevention of perioperative tachyarrhythmia. We undertook this study to examine possible preventive effects of dexmedetomidine on postoperative JET and its impact on the duration of ventilation time and length of Intensive Care Unit stay. Methods: After obtaining approval from the hospitals ethics committee and written informed consent from parents, this quasi-randomized trial was initiated. Of 94 patients, 47 patients received dexmedetomidine (dexmedetomidine group) and 47 patients did not receive the drug (control group). Results: Dexmedetomidine group had more number of complex variants like TOF with an absent pulmonary valve or pulmonary atresia (P = 0.041). Hematocrit on cardiopulmonary bypass (CPB), heart rate while coming off from CPB and inotrope score was significantly low in the dexmedetomidine group compared to control group. The incidence of JET was significantly low in dexmedetomidine group (P = 0.040) compared to control group. Conclusions: Dexmedetomidine may have a potential benefit of preventing perioperative JET.

Tetralogy of Fallot in a Case of Dextrocardia with Situs Solitus.

Dextrocardia associated with situs solitus (so called ‘dextroversion’) is a cardiac positional anomaly in which the heart is located in the right hemithorax with its base-toapex axis directed to the right and caudad along with normal anatomical position of other intrathoracic and abdominal viscera. The malposition is intrinsic to the heart and not caused by extracardiac abnormalities. Dextroversion is the second most common type of dextrocardia. In dextroversion, there is a 90% incidence of additional cardiac malformations, including anomalous pulmonary venous return, Tetralogy of Fallot, septal defects, pulmonic stenosis, coarctation of the aorta, and corrected TGA.Here we report a rare case of Tetralogy of Fallot in a 17 year old male with dextroversion.

Análisis de microdeleciones en 22q11 en pacientes colombianos con cardiopatía congénita no sindrómica / Analysis of microdeletions in 22q11 in Colombian patients with congenital heart disease

Los defectos cardiacos conforman las malformaciones congénitas más frecuentes, con una incidencia que se ha estimado entre 4 y 12 por 1000 en recién nacidos vivos. Estos tienen una etiología multifactorial en la que convergen la predisposición genética y los factores ambientales. A partir de 1990 se ha relacionado este tipo de patologías con microdelección 22q11. Se determinó la frecuencia de la microdeleción 22q11 en pacientes con cardiopatía congénita no sindrómica. Se analizaron 61 pacientes con cardiopatía congénita, a partir de ADN de sangre periférica y posterior amplificación, mediante PCR multiplex del gen TUPLE1 y del STR D10S2198, visualización electroforesis en geles de agarosa y análisis densitométrico para determinar dosis génica. Se encontraron 3 pacientes con microdeleción 22q11, para una frecuencia de 4,9%. Esta microdeleción se asoció en dos de los casos a Tetralogía de Fallot y en el otro a Defecto Septal Atrial (DSA). En conclusión, la frecuencia de microdeleción 22q11 en la población analizada es de 4,9%. Dentro de los casos de Tetralogía de Fallot, la microdeleción estaba presente en el 7,4% y en los DSA corresponde al 11,1%.

Cardiac defects are the most frequent congenital malformations, with an incidence estimated between 4 and 12 per 1000 newborns. Their etiology is multifactorial and might be attributed to genetic predispositions and environmental factors. Since 1990 these types of pathologies have been associated with 22q11 microdeletion. In this study, the frequency of microdeletion 22q11 was determined in 61 patients with non-syndromic congenital heart disease. DNA was extracted from peripheral blood and TUPLE1 and STR D10S2198 genes were amplified by multiplex PCR and visualized in agarose gels. Gene content was quantified by densitometry. Three patients were found with microdeletion 22q11, representing a 4.9% frequency. This microdeletion was associated with two cases of Tetralogy of Fallot and a third case with atrial septal defect (ASD). In conclusion, the frequency for microdeletion 22q11 in the population analyzed was 4.9%. The cases that presented Teratology of Fallot had a frequency for this microdeletion of 7.4% and for ASD of 11.1%.

Anatomical variations of pulmonary artery and associated cardiac defects in tetralogy of Fallot

To determine pulmonary artery variations and other associated cardiac defects in patients with Tetralogy of Fallot [TOF]. Cross-sectional, descriptive study. The Children's Hospital and the Institute of Child Health, Lahore, from April 2006 to October 2007. All patients with TOF, who underwent cardiac catheterization during this period, were included. Standard cine-angiograms were recorded and pulmonary artery sizing was done using z-scoring. A total of 216 patients with TOF were catheterized. Pulmonary Artery [PA] abnormalities were present in 84 [38.9%] patients. The commonest abnormality was isolated Left Pulmonary Artery [LPA] stenosis [n=27, 32.14%] followed by isolated hypoplasia of Main Pulmonary Artery [MPA] [n=18, 21.43%] and supra-valvular stenosis in [n=11, 13.1%] patients. LPA was absent in one patient, while 2 patients had both absent right and left PA with segmental branch pulmonary arteries originating directly from MPA. Associated cardiac lesions included right aortic arch in 34 [15%], additional muscular VSD vary in 13 [5.5%], Patent Ductus Arteriosus [PDA] in 11 [6%] and Major Aortopulmonary Collateral Arteries [MAPCA] in 2 [1.9%] patients. Significant coronary artery abnormality was present in 10 [4.6%] children. Pulmonary artery abnormalities were present in 38.9% of patients with TOF. Isolated LPA origin stenosis and MPA hypoplasia were the most common abnormalities. Significant associated cardiac lesions were present in one-third of the patients and included PDA, additional muscular VSD, coronary artery abnormalities and MAPCA

Prevalence of congenital heart disease, Kanpur, India.

Our aim was to study the prevalence, age-wise distribution, and clinical spectrum of congenital heart disease (CHD) at a multi-speciality corporate hospital in North India. A retrospective analysis of records of 10,641 patients over a five-and-a-half year period was done. Clinical examination, echocardiography and color doppler were used as diagnostic tools. A prevalence of 26.4 per 1000 patients was observed. VSD (ventricular septal defect) was the commonest lesion (21.3%), followed by ASD (atrial septal defect) in 18.9% and PDA (patent ductus arteriosus) in 14. 6%. Tetralogy of Fallot was the commonest cyanotic heart disease (4.6%). Maximum number of children with heart disease (82.9%) were diagnosed between 0-3 years of age.

Treinta y tres años de cirugía correctora en tetralogía de Fallot / Surgical results in patients with tetralogy of Fallot

En 1957 se inició en el Centro Cardiovascular del Hospital "Luis Calvo Mackenna" la corrección quirúrgica de la tetralogía de Fallot (excluidos los niños con atresia pulmonar y comunicación interventricular). Desde entonces hasta diciembre de 1989 se han intervenido 591 de estos pacientes. Se revisa la letalidad entre los 92 y 243 pacientes operados desde 1957 a 1967 y desde 1968 a 1979, respectivamente, así como en los 256 pacientes del período 1980 a 1989, en los que también se analizan las complicaciones postoperatorias y los resultados a corto y mediano plazo (seguimiento promedio 57,5 meses), según edad y técnica quirúrgica empleada. La letalidad general ha descendido de 32,6% en el primer período a 4,29% entre los años 1980 y 1989, en que no fallecieron pacientes corregidos primariamente antes del año de vida (17 casos). No hubo diferencias significativas de letalidad según técnicas empleadas (parche infundibular, parche transanular, vía transanular o valvulotomía exclusiva). La principal causa de muerte fue bajo débito cardíaco en el período postoperatorio (82%). En 98,8% de los pacientes del último período de estudio la capacidad funcional actual es I (NYHA). Sólo un paciente tiene evidencia de insuficiencia severa de válvula pulmonar. Existe una clara tendencia a hacer reparaciones más precoces y notoria disminución de la letalidad, requiriéndose mayores estudios prospectivos de largo plazo, para evaluar morbilidad futura de las distintas técnicas actualmente empleadas