Definisi Tetralogi fallot (TF) adalah kelainan jantung dengan gangguan sianosis yang ditandai dengan kombinasi 4 hal yang abnormal meliputi. Tetralogy of Fallot is a congenital cardiac malformation that consists of an interventricular communication, also known as a ventricular septal defect, obstruction. BAB II TETRALOGY OF FALLOT A. REVIEW ANATOMI FISIOLOGI JANTUNG Kelainan sianotik memiliki pengertian bahwa bayi mempunyai.
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Home Documents Tetralogi Fallot – Bahan. Post on Nov 65 views. Most patients, however, require repeat catheterizations for balloon dilation or stent placements in stenotic pulmonary artery segments to alleviate elevated right ventricular pressures. Pediatric cardiology consultation is advised. Consult a geneticist to evaluate the presence tetralohi syndromic associations and gene deletions, especially in the presence of associated anomalies or dysmorphic features.
Konsep-Askep Tetralogi of Fallot
Once the anatomy of a child with TOF-PA is determined by echocardiography and angiography findings, consultation with a cardiovascular surgeon is required.
The caregivers need to be aware of the possibility of a multistage repair and repeated surgeries and catheterizations. If anomalies involving other systems are present, consultations and follow-up with the appropriate specialists are required.
Infants who are born with multiple systemic-to-pulmonary collaterals and are in cardiac failure because of pulmonary overcirculation require caloric supplementation to establish a normal growth pattern. Children that undergo palliative procedures also require optimization of their caloric intake. Adequate nutritional supplementation in the form of total parental nutrition must also be ascertained in the perioperative period.
These patients often have a prolonged postoperative recovery course. Exercise tolerance and need for restrictions on physical activity depend on the type of repair and hemodynamic state of the patient. Patients with cyanosis will have significantly limited exercise capacity. A PGE1 Alprostadil infusion maintains patency of the ductus. Infants with multiple systemic pulmonary collaterals may develop symptomatic heart failure requiring medical therapy. Prostaglandins — PGE1 Alprostadil promotes dilatation of the DA in infants withductal-dependent cardiac abnormalities.
It is also a vasodilator. Beneficial in infants who have congenital defects that restrict pulmonary or systemic blood flow and who depend on a patent DA for adequate oxygenation and lower body perfusion. Produces vasodilation and increases cardiac output.
Diuretic agents — These agents promote excretion of water and electrolytes bythe kidneys. They are used to treat heart failure or hepatic, fsllot, or pulmonary disease when sodium and water retention results in edema or ascites. Children who have CHF symptoms often require multiple diuretics for effective control. Furosemide Lasix — Increases excretion of defiisi by interfering with chloride-binding cotransport system, which in turn inhibits sodium and chloride reabsorption in ascending loop of Henle and distal renal tubule.
Individualize dose to patient. Depending on response, administer adult doses at increments of mg, no sooner than h after previous dose, until desired diuresis occurs. Perform frequent serum electrolyte, CO2, glucose, creatinine, uric acid, calcium, and BUN determinations during first few months of therapy and periodically thereafter Spironolactone Aldactone — For management of edema dffinisi from excessive aldosterone excretion.
Competes with aldosterone for receptor sites in distal renal tubules, increasing water excretion while retaining potassium and hydrogen ions. Inotropic agents — Positive inotropic agents increase the force of contraction ofthe myocardium and are used to treat acute and chronic CHF. Poor ventricular function may necessitate the use falkot inotropic medications.
Digoxin Lanoxin — Cardiac glycoside with direct inotropic effects and indirect effects on the cardiovascular system. Falot directly on cardiac muscle, increasing myocardial systolic contractions. Indirect actions result in increased carotid sinus nerve activity and enhanced sympathetic withdrawal for any given increase in mean arterial pressure.
Hypokalemia may reduce positive inotropic effect of digitalis; hypercalcemia predisposes patient to digitalis toxicity, and hypocalcemia can make digoxin ineffective until serum calcium levels are within tetralgoi reference range; magnesium replacement therapy must be instituted in patients with hypomagnesemia to prevent digitalis toxicity; incomplete AV block may progress to complete block when treated withInteractionsPregnancy Precautionsdigoxin; exercise caution in hypothyroidism, hypoxia, and acute myocarditis; adjust dose in renal impairment; highly toxic overdoses can be fatal FOLLOW-UPSection 8 of 11Author Information Introduction Clinical Differentials Workup Treatment Medication Follow-up Miscellaneous Pictures BibliographyFurther Inpatient Care: Admit for testing and surgical intervention.
Significant pulmonic valve regurgitation often occurs regardless of the type of conduit placed between the right ventricle and the pulmonary arteries. Some patients develop substantial right ventricular dilation and right ventricular dysfunction. Surgical placement of a pulmonic valve may significantly benefit these patients. Transcatheter placement of a pulmonic valve currently is under development.
Infants with multiple aortopulmonary collaterals may require outpatient medical management of heart failure. Residual right ventricular hypertension with right ventricular dysfunction from hypoplastic pulmonary arteries may be present.
After each stage of surgical reconstruction, echocardiographic and Doppler evaluation of hemodynamic adequacy should be performed. After complete repair, the faplot needs to be evaluated for the development of right ventricletopulmonary artery conduit stenosis as well as pulmonic regurgitation. A few patients may never reach the stage of complete repair because of very hypoplastic pulmonary arteries.
These patients often are hypoxemic and polycythemic and may require oxygen supplementation. Transfer to a tertiary care center is indicated for complete diagnostic evaluation and surgical intervention. Residual right ventricular dysfunction from hypoplastic pulmonary arteries or conduit stenosis Tstralogi, hypoxemia, and polycythemia Atrioventricular conduction abnormalities, right bundle branch block, ventricular arrhythmias in the postoperative patients Significant pulmonic valve regurgitationPrognosis: The prognosis depends on the specific anatomy and type of intervention.
Long-term follow up data are not widely available; however, recent outcome does seem to be more favorable.
Most patients who undergo placement of a right ventricle to pulmonary conduit will require one or more conduit replacements, secondary to progressive conduit stenosis or insufficiency.
Educate patients and their families fal,ot anatomic details and long-term prognosis, the potential need for multiple surgeries and catheterizations, and postoperative complications. At all patient care visits, emphasize the need for bacterial endocarditis prophylaxis. For excellent patient education resources, visit eMedicine’s Heart Center.
Also, see eMedicine’s patient education article Tetralogy of Fallot.
Failure to consider the diagnosis, especially in a newborn with cyanosisSpecial Concerns: Patients with residual right ventricular dysfunction or pulmonary hypertension are advised to avoid pregnancy because it carries significant mortality risk. All patients with TOF-PA are required to take appropriate antibiotic bacterial endocarditis prophylaxis. Exercise recommendations must be tailored to individual patients by considering the presence of cyanosis, right ventricle hypertension, right ventricle dysfunction, or dysrhythmias.
Parasternal long axis two-dimensional echocardiographic image demonstrating a large malalignment ventricular septal defect with overriding of the aorta over the ventricular septum. Subcostal sagittal plane two-dimensional echocardiographic image showing pulmonary valve atresia, with confluent and well-developed pulmonary artery branches. Suprasternal long axis color flow echocardiographic image showing a large patent ductus arteriosus supply confluent pulmonary arteries.
Aortopulmonary view angiogram, with injection in the descending thoracic aorta demonstrating multiple aortopulmonary collaterals supplying pulmonary blood flow. Parasternal long axis two-dimensional echocardiographic image in a patient status post complete repair of tetralogy of Fallot with pulmonary atresia. A patch is visualized closing the ventricular septal defect.
Parasternal long axis color compare echocardiographic image showing the pulmonary artery conduit arising from the right ventricle. Tetralogy of Fallot TOF is a complex of anatomic abnormalities arising from the maldevelopment of the right ventricular infundibulum. InFallot described the anatomy as consisting of a subaortic ventricular septal defect VSDright ventricular infundibular stenosis, aortic valve positioned to override the right ventricle, and right ventricular hypertrophy RVH.
See Image 1 for a schematic illustration of these abnormalities. Wide variation exists in the basic anatomic morphology, pathophysiology, clinical signs and symptoms, and surgical methods of therapy.
Pathophysiology is dependent primarily upon severity of the right ventricular outflow tract RVOT obstruction. Right-to-left shunting is typical. Natural history is variable. Natural history is determined mainly by the degree of RVOT obstruction. Incidence is slightly higher in males than females.
Cyanosis develops within the first few years of life. First presentation may include poor feeding, fussiness, tachypnea, and agitation. Symptoms generally progress secondary to hypertrophy of the infundibular septum. Cyanosis occurs and demands surgical repair. Dyspnea on exertion is common. Squatting is uniquely characteristic of a right-to-left shunt that presents in the exercising child. Hypoxic “tet” spells are potentially lethal, unpredictable episodes that occur even in noncyanotic patients with TOF.
These spells can be aborted with relatively simple procedures. The rare patient may remain marginally and imperceptibly cyanotic, or acyanotic and asymptomatic, into adult life. Severe cyanosis may present at birth in a patient with TOF and associated pulmonary atresia. Birth weight is low. Development and puberty may be delayed.
Right ventricular predominance on palpation May have a bulging left hemithorax Systolic thrill at the lower left sternal border Aortic ejection click Single S2 – Pulmonic valve closure not heard Systolic ejection murmur – Varies in intensity inversely with the degree of RVOT obstructiono oMore cyanotic patients have greater obstruction and a softer murmur.
As one of the conotruncal malformations, TOF can be associated with a spectrum of lesions known as CATCH 22 cardiac defects, abnormal facies, thymic hypoplasia, cleft palate, hypocalcemia.
Tetralogi Fallot – Bahan – [DOC Document]
Cytogenetic analysis may demonstrate deletions of a segment of chromosome band 22q11 DiGeorge critical region. Ablation of cells of the neural crest has been shown to reproduce conotruncal malformations.
falot These abnormalities are associated with the DiGeorge syndrome and branchial arch abnormalities. TOF frequently is associated with the following: Ebstein malformation of the tricuspid valve Pulmonary atresia Ventricular septal defect Pseudotruncus arteriosus Lab Studies: Oximetry and arterial blood gasesoOxygen saturation is variable, but pH and pCO2 are normal unless the patient is in extremis, such as during a tet spell.
Oximetry is particularly useful in the dark-skinned patient or the anemic patient whose level of cyanosis is not apparent. A decrease in systemic vascular resistance SVR during exercise, bathing, or fever potentiates a right-to-left shunt and causes hypoxemia. Hyperviscosity and coagulopathy often ensue and are particularly deleterious in patients tetrallogi a right-to-left intracardiac shunt. Stroke and brain abscess are natural corollaries.
Blow-by O2 BBO2 is the least objectionable.