Which of the following in the natural course of disease has no reversal of the shunt?
Correct Answer: TOF
Description: Ans. c. TOF (Ref. Myong K. Park 5/e p238)Natural History of TOF: (Myong K. Park 5/e p238)Infants with acvanotic gradually become cyanotic as a result of worsening condition of the infundinular stenosis and polycythemiaPolycythemia develop secondary to cyanosisHypoxic spells may develop in infantsGrowth retardation may be present if cyanosis is severeBrain abscess and cerebrovascular accidents rarely occurSABE is occasionally a complicationSome patients, particularly those with severe TOF develop ARCoagulopathy is a late complication of longstanding cyanosisEisenmenger syndromeEisenmenger syndrome was so named by Dr. Paul Wood after Dr. Victor Eisenmenger, who first described the condition in 1897.Congenital heart defects causing Eisenmenger syndromeAtrial septal defectsVentricular septal defectsPatent ductus arteriosusMore complex types of acyanotic heart disease.Calcium chloride is not given in the treatment of cyanotic spells in a patient of TOF.Management of Anoxic Spells in Tetralogy of FallotKnee chest positionHumidified oxygenMorphineQ 0.1 to 0.2 mg/kg SCObtain venous pH; sodium bicarbonateQ 1-3 ml/kg (diluated) IVPropranolol 0.1 mg/kg/IV (during spell)Q; 0.5-1 mg/kg/6 hourly orally (alternatives: metoprolol, esmoiol)Vasopressors: Methoxamine (Vasoxy1) IM or IV drip (Phenylephrine is a vasopressor)QCorrect anemiaConsider surgeryTetralogy of FallotCommonest congenital cyanotic congenital heart diseaseQ in children above the age of 2 years constituting almost 75 percent of all blue patients.Hemodynamics:Physiologically the pulmonary stenosis causes concentric right ventricular hypertrophy without cardiac enlargementQ and an increase in right ventricular pressure.Severity of cyanosis is directly proportional to the severity of pulmonic stenosis, but the intensity of the systolic murmur is inversely proportional to the severity of pulmonic stenosisQ.Since the right ventricle is e.ffectively decompressed by the ventricular septal defect, congestive failure never occurs in TOFQ.The late and soft P2 is generally inaudible in TOF. The S2 is therefore single and the audible sound is A2.On auscultation, the diastolic interval is completely clear in TOF as there is no third or fourth sound or diastolic murmur.Clinical Features:MC symptoms are dyspnoea on exertion and exercise intoleranceQ.Squatting is not specific for TOF, TOF is the commonest congenital lesion in which squatting is notedQ.Characteristic features:Normal sized heart with upturned apexQ (suggestive of RVH)Absence of main pulmonary artery segment gives it the shape described as 'Cor-en Sabot'Q Pulmonary fields are oligaemicQAortic-mitral valve continuity is maintainedQ.Complications:Anemia, infective endocarditis, venous thrombosisParadoxical embolism, hemiplegia, brain abscessQTreatment:Medical management for anemia and management of complications.Palliative operations in TOF1. Blalock-Taussig shunt: Subclavian artery-pulmonary artery anastomosisQ.2. Potts shunt: Descending aorta is anastomosed to pulmonary arteryQ.3. Waterston's shunt: Ascending aorta-right pulmonary artery anastomosisQ.Definitive operations: Closing the VSD and resecting the infundibular obstructionQ.Fallot'sTrilogyTetralogyPentalogy* Pulmonary stenosisQ* Atrial septal defectQ* Right ventricular hypertrophQ* Pulmonary stenosisQ* Ventricular septal defectQ* Right ventricular hypertrophyQ* Ovemding or dextroposed aortaQ.* Tetralogy of Fallot + ASDQ or patent foramen ovaleQ
Category:
Pediatrics
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