You are on page 1of 11

Prognosis Bayi dengan bentuk TOF yang terberat biasanya mengalami sianosis berat pada saat kelahiran.

Hipoxia spell mungkin terjadi selama periode neonatal. Kematian secara ekstrem jarang selama hypoxia spell yang berat. Anak-anak dengan kondisi ini memerlukan operasi segera, baik Blalock-Taussig shunt maupun koreksi primer. Semua pasien dengan TOF memerlukan operasi jantung terbuka. Perbaikan komplit sebelum usia 5 tahun biasanya menghasilkan fungsi yang bagus, meskipun pasien biasanya meninggal akibat disritmia ventrikel. Katup pulmonary kompeten tanpa pembesaran ventrikel kanan tampaknya mampu mengurangi aritmia dan meningkatkan exercise performance (Hay Jr., 2002). Terapi Terapi dari TOF bergantung pada tingkat keparahan obstruksi aliran keluar ventrikel kanan. Bayi dengan TOF parah memerlukan perawatan medis dan intervensi bedah pada periode neonatal. Terapi ini bertujuan memberikan peningkatan langsung aliran darah paru untuk mencegah sequelae hipoksia berat. Bayi harus dibawa ke pusat medis memadai untuk mengevaluasi dan mengobati neonatus dengan penyakit jantung bawaan di bawah kondisi optimal. Sangat penting bahwa oksigenasi dan suhu tubuh normal dipertahankan selama transportasi. Hipoksia yang berat dan lama bisa menyebabkan syok, kegagalan pernapasan, dan asidosis dan secara signifikan akan mengurangi kesempatan untuk bertahan hidup. Suhu dingin meningkatkan konsumsi oksigen, yang memberikan stress tambahan pada bayi sianosis. Kadar glukosa darah harus dipantau karena hipoglikemia lebih mungkin terjadi pada bayi dengan penyakit jantung sianosis. Bayi dengan obstruksi aliran keluar ventrikel kanan dapat memburuk dengan cepat karena ketika duktus arteriosus mulai menutup, aliran darah paru lebih lanjut compromised. Pemberian prostaglandin E1 intravena (0,05-0,20 mg/kg/min) menyebabkan dilatasi duktus arteriosus dan biasanya menyediakan aliran darah paru yang memadai sampai prosedur pembedahan dapat dilakukan. Agen ini harus diberikan segera setelah penyakit jantung sianosis bawaan secara klinis dicurigai dan terus diberikan selama periode pra operasi dan kateterisasi jantung. Pasca operasi, infus dapat dilanjutkan secara singkat sebagai vasodilator pulmonal untuk menambah aliran melalui shunt paliatif atau melalui valvulotomy bedah.

Bayi dengan obstruksi aliran keluar ventrikel kanan yang kurang parah, stabil, dan menunggu intervensi bedah mengharuskan adanya observasi yang cermat. Pencegahan atau pengobatan segera terhadap dehidrasi penting untuk menghindari hemokonsentrasi dan episode trombotik. Serangan dispneu paroksismal pada bayi atau awal masa kanak-kanak dapat dipicu oleh defisiensi Fe relatif; pemberian Fe dapat menurunkan frekuensi dan juga meningkatkan toleransi latihan dan keadaan umum. Indeks sel darah merah harus dipertahankan dalam kisaran normositik. Propranolol oral (0.5-1 mg/kg setiap 6 jam) dapat menurunkan frekuensi dan keparahan hipercyanotic spell, tetapi dengan operasi yang tersedia, pengobatan bedah diindikasikan sesegera setelah hipoxia spell terjadi. Bayi dengan sianosis parah pada 1 bulan pertama kehidupan menunjukkan adanya obstruksi airan keluar ventrikel kanan atau atresia paru. Dua pilihan terapi yang tersedia pada bayi ini: yang pertama adalah shunt paliatif sistemik ke paru dilakukan untuk meningkatkan aliran darah arteri pulmonal. Alasan operasi ini adalah untuk mengurangi jumlah hipoksia dan meningkatkan pertumbuhan linear, serta meningkatkan pertumbuhan cabang arteri paru. Pilihan kedua adalah operasi korektif jantung terbuka dilakukan pada awal masa bayi dan bahkan pada masa neonatus pada bayi sakit kritis. Pada bayi dengan sianosis yang kurang berat yang dapat dipertahankan dengan pertumbuhan yang baik dan tidak adanya hypercyanotic spells, perbaikan primer dilakukan secara elektif di antara usia 4 dan 12 bulan. Shunt Blalock-Taussig yang dimodifikasi merupakan prosedur shunt aortopulmonary yang paling umum saat ini dan terdiri atas saluran Gore-Tex dianastomosikan sisi ke sisi dari arteri subklavia ke cabang homolateral dari arteri pulmonalis. Kadang-kadang saluran yang dibawa langsung dari aorta ascenden ke arteri pulmonalis utama dan disebut shunt pusat. Operasi Blalock-Taussig dapat dilakukan pada periode bayi baru lahir dengan shunt 3-4 mm dan juga telah berhasil digunakan pada bayi prematur. Komplikasi pasca operasi dapat terjadi setelah torakotomi lateral dan termasuk chylothorax, kelumpuhan diafragma, dan sindrom Horner. Chylothorax mungkin memerlukan thoracocentesis berulang dan kadang-kadang membutuhkan reoperasi untuk meligasi duktus thoracicus. Dukungan ventilator berkepanjangan dan terapi fisik yang kuat mungkin diperlukan pada kelumpuhan diafragma, tetapi fungsi diafragma biasanya kembali dalam 1-2 bulan.. Sindrom Horner biasanya bersifat sementara dan tidak memerlukan pengobatan. Gagal jantung pasca operasi dapat disebabkan oleh shunt besar. Setelah prosedur shunt sukses, sianosis akan berkurang. Terdengarnya murmur kontinyu pada lapangan paru setelah operasi menunjukkan berfungsinya anastomose berfungsi. Murmur shunt yang baik mungkin tidak terdengar sampai beberapa hari setelah

operasi. Durasi penyembuhan gejala bervariasi. Seiring dengan pertumbuhan anak tumbuh, maka semakin besar aliran darah paru yang dibutuhkan dan shunt akhirnya menjadi inadekuat. Ketika sianosis bertambah berat, terapi bedah korektif harus dilakukan jika secara anatomis memungkinkan. Jika tidak mungkin (misalnya, karena cabang arteri pulmonalis hipoplasia) atau jika shunt pertama hanya bertahan sebentar pada bayi kecil, anastomose aortopulmonary kedua mungkin diperlukan pada sisi yang berlawanan. Terapi bedah korektif dilakukan dengan menghilangkan obstruksi aliran keluar ventrikel kanan dengan membuang bundel otot obstruktif dan penutupan patch VSD. Jika katup pulmonal mengalami stenosis, valvotomi yang dikerjakan. Jika anulus katup pulmonal kecil atau katup sangat menebal, valvectomy mungkin dilakukan, anulus katup pulmonal dibelah, dan patch transannular ditempatkan pada cincin katup pulmonal. Setiap shunt sistemik ke paru yang dibuat sebelumnya harus danihilang sebelum perbaikan penuh. Risiko operasi koreksi total kurang dari 5%. Sebuah ventriculotomy yang tepat merupakan pendekatan standar, tetapi pendekatan transatrial-transpulmonary dapat digunakan untuk mengurangi risiko jangka panjang dari suatu ventriculotomy. Setelah koreksi keseluruhan berhasil, pasien umumnya tidak bergejala. Masalah pasca operasi segera termasuk kegagalan ventrikel kanan, blok jantung sementara, VSD residual dengan shunt kiri-ke-kanan, infark miokard dari gangguan arteri koroner aberrant, dan peningkatan tekanan atrium kiri disproporsional karena kolateral bronkial residual. Gagal jantung pasca operasi (terutama pada pasien dengan patch transannular) membutuhkan agen inotropik positif seperti digoksin. Sebagian besar pasien setelah perbaikan TOF dan semua dengan perbaikan patch transannular yang memiliki murmur to-and-fro pada perbatasan sternum kiri, biasanya menunjukkan adanya obstruksi aliran keluar ringan dan insufisiensi pulmonal ringan sampai sedang. Pasien dengan insufisiensi katup pulmonal yang jelas juga memiliki pembesaran jantung moderat. Tindak lanjut dari pasien 5-20 tahun setelah operasi menunjukkan bahwa perbaikan gejala yang pada umumnya dapat dipertahankan. Pasien asimtomatik memliki kapasitas latihan normal, denyut jantung maksimal, dan output jantung yang lebih rendah. Temuan abnormal ini lebih umum pada pasien yang menjalani pemasangan patch transannular dan mungkin kurang sering ketika operasi dilakukan pada usia dini. Gangguan konduksi dapat terjadi setelah operasi. Nodus AV dan berkas His dan divisinya berada di dekat VSD dan mungkin mengalami jejas selama operasi. Blok jantung total permanen setelah operasi jarang terjadi. Ketika hal tersebut ditemukan, maka harus diatasi dengan penempatan pacemaker implant yang permanen. Bahkan blok jantung total transien

pada periode pasca operasi segera jarang terjadi pada pasien TOF. Hal itu mungkin berhubungan dengan peningkatan insiden blok jantung total onset akhir dan kematian mendadak. Right bundle branch block cukup umum pada elektrokardiogram pasca operasi. Durasi interval QRS mampu memprediksi baik kehadiran kekacauan hemodinamik residual dan risiko jangka panjang kematian mendadak. Sejumlah anak-anak memiliki denyut prematur ventrikel setelah perbaikan TOF. Gangguan ini menjadi perhatian terutama pada pasien dengan kelainan hemodinamik residual; studi pemantauan 24-jam elektrokardiografi (Holter) harus dilakukan untuk memastikan bahwa episode pendek ventricular takikardia tidak terjadi. Dengan adanya aritmia ventrikel yang kompleks atau kelainan hemodinamik residual yang parah, terapi antiarrhythmic profilaksis dapat dibenarkan. Perbaikan ulang diindikasikan jika didapatkan obstruksi aliran keluar ventrikel kanan residual yang signifikan atau insufisiensi pulmonal berat. (Nelson, 2003). Treatment A. PALLIATIVE TREATMENT Palliative treatment is performed at some centers for small infants who are markedly symptomatic (severely cyanotic, frequent severe hypoxic spells) and in whom complete correction is deemed to be too risky. Medical (chronic oral b-blocking agents) or, more often, surgical (creation of a systemic arterial to pulmonary arterial anastomosis) palliation can be used. In addition, balloon angioplasty, to dilate the pulmonic valve and the entire right ventricular outflow tract in order to reduce right ventricular outflow tract obstruction and improve pulmonary artery growth, is now being used as palliation in some centers. The most common surgical palliation is the creation of a Gore-Tex shunt from the subclavian artery to the ipsilateral pulmonary artery (modified Blalock-Taussig shunt). This operation can be done with very low mortality and with no significant distortion of the pulmonary arteries. B. TOTAL CORRECTION The timing of total correction ranges from birth to age 2 years, varying with the philosophies and results of each treatment center. It involves closing the VSD and removing the obstruction to right ventricular outflow. The surgical death rate varies from 1% to 3%. The major limiting anatomic feature of total correction is the size of the pulmonary arteries. HYPOXIC SPELL

Hypoxic spell (also called cyanotic spell, hypercyanotic spell, tet spell) of TOF requires immediate recognition and appropriate treatment, because it can lead to serious complications of the central nervous system. Hypoxic spells are characterized by a paroxysm of hyperpnea (i.e., rapid and deep respiration), irritability and prolonged crying, increasing cyanosis, and decreasing intensity of the heart murmur. Hypoxic spells occur in infants, with a peak incidence between 2 and 4 months of age. These spells usually occur in the morning after crying, feeding, or defecation. A severe spell may lead to limpness, convulsion, cerebrovascular accident, or even death. There appears to be no relationship between the degree of cyanosis at rest and the likelihood of having hypoxic spells ( see Chapter 11 ). Treatment of the hypoxic spell strives to break the vicious circle of the spell ( see Fig. 11-9 ). Physicians may use one or more of the following to treat the spell. 1. The infant should be picked up and held in a knee-chest position. 2. Morphine sulfate, 0.2 mg/kg administered subcutaneously or intramuscularly, suppresses the respiratory center and abolishes hyperpnea. 3. Oxygen is usually administered, but it has little demonstrable effect on arterial oxygen saturation. 4. Acidosis should be treated with sodium bicarbonate (NaHCO3 ), 1 mEq/kg administered intravenously. The same dose can be repeated in 10 to 15 minutes. NaHCO3 reduces the respiratory center-stimulating effect of acidosis. With the preceding treatment, the infant usually becomes less cyanotic, and the heart murmur becomes louder, which indicates an increased amount of blood flowing through the stenotic right ventricular outflow tract. If the hypoxic spells do not fully respond to these measures, the following medications can be tried: 1.

Vasoconstrictors

such

as

phenylephrine

(Neo-Synephrine),

0.02

mg/kg

administered

intravenously, may be effective. 2. Ketamine, 1 to 3 mg/kg (average of 2 mg/kg) administered intravenously over 60 seconds, works well. It increases the systemic vascular resistance and sedates the infant. 3. Propranolol, 0.01 to 0.25 mg/kg (average 0.05 mg/kg) administered by slow intravenous push, reduces the heart rate and may reverse the spell. MANAGEMENT Medical 1. Physicians should recognize and treat hypoxic spells ( see the preceding section and Chapter 11 ). It is important to educate parents to recognize the spell and know what to do. 2. Oral propranolol therapy, 0.5 to 1.5 mg/kg every 6 hours, is occasionally used to prevent hypoxic spells while waiting for an optimal time for corrective surgery in countries where openheart surgical procedures are not well established for small infants,

* This occurs in all types of cyanotic congenital heart defects. 194 3. Balloon dilatation of the right ventricular outflow tract and pulmonary valve, though not widely practiced, has been attempted to delay repair for several months. 4. Maintenance of good dental hygiene and practice of antibiotic prophylaxis against SBE are important ( see Chapter 19 ).

5. Relative iron-deficiency anemia should be detected and treated. Anemic children are more susceptible to cerebrovascular complications. Normal hemoglobin or hematrocrit values or decreased red blood indices indicate an iron-deficiency state in cyanotic patients. Surgical Palliative Shunt Procedures Indications. Shunt procedures are performed to increase PBF. Indications for shunt procedures vary from institution to institution. Many institutions, however, prefer primary repair without a shunt operation regardless of the patient's age. Selected indications for shunt procedures follow: 1. Neonates with TOF and pulmonary atresia. 2. Infants with hypoplastic pulmonary annulus, which requires a transannular patch for complete repair. 3. Children with hypoplastic PAs. 4. Severely cyanotic infants younger than 3 months of age. 5. Infants younger than 3 to 4 months old who have medically unmanageable hypoxic spells. Procedures, Complications, and Mortality. Although other procedures were performed in the past ( Fig. 14-20 ), only Blalock-Taussig and Gore-Tex interposition shunt (i.e., modified Blalock-Taussig) procedures are performed at this time. They have a surgical mortality rate of 1% or less. 1.

Classic Blalock-Taussig shunt, anastomosed between the subclavian artery and the ipsilateral PA, is usually performed for infants older than 3 months ( see Fig. 14-20 ). A right-sided shunt is performed in patients with left aortic arch; a left-sided shunt is performed for right aortic arch. 2. Gore-Tex interposition shunt, placed between the subclavian artery and the ipsilateral PA, is the procedure of choice for small infants younger than 3 months of age and sometimes for older infants ( see Fig. 14-20 ). A left-sided shunt is preferred for patients with left aortic arch, whereas a right-sided shunt is preferred for patients with a right aortic arch. 3. The Waterston shunt, anastomosed between the ascending aorta and the right PA, is no longer performed because of a high incidence of surgical complications ( see Fig. 14-20 ). 195 Complications resulting from this procedure included too large a shunt leading to CHF and/or pulmonary hypertension, and narrowing and kinking of the right PA at the site of the anastomosis. The latter created difficult problems in closing the shunt and reconstructing the right PA at the time of corrective surgery. 4. The Potts operation, anastomosed between the descending aorta and the left PA, is no longer performed either ( see Fig. 14-20 ). It may result in heart failure or pulmonary hypertension, as in the Waterston operation. A separate incision (i.e., left thoracotomy) is required to close the shunt during corrective surgery, which is performed through a midsternal incision. Conventional Repair Surgery. Timing of this operation varies from institution to institution, but early surgery is generally preferred. Indications and Timing 1. Symptomatic infants who have favorable anatomy of the right ventricular outflow tract and PAs may have primary repair at any time after 3 to 4 months of age. Some centers perform primary repair in younger infants and newborns, with an early mortality rate of <5%. Advantages cited

for early primary repair include diminution of hypertrophy and fibrosis of the RV, normal growth of the PAs and alveolar units, and reduced incidence of postoperative ventricular ectopic beats and sudden death. 2. Asymptomatic and minimally cyanotic children may have the repair between 3 and 24 months of age, depending on the degree of annular and PA hypoplasia. 3. Mildly cyanotic infants who have had previous shunt surgery may have total repair 1 to 2 years after the shunt operation. 4. Asymptomatic and acyanotic children (i.e., pink tet) have the operation at 1 to 2 years of age. 5. Asymptomatic children with coronary artery anomalies may have the repair at 3 to 4 years of age, because a conduit placement may be required between the RV and the PA. Procedure. Total repair of the defect is carried out under cardiopulmonary bypass and circulatory arrest. The procedure includes patch closure of the VSD and widening of the right ventricular outflow tract by resection of the infundibular tissue and placement of a fabric patch ( Fig. 14-21 ). Figure 14-21 Total correction of tetralogy of Fallot (TOF). A, Anatomy of TOF showing a large ventricular septal defect (VSD) and infundibular stenosis through a right ventriculotomy. B, Patch closure of the VSD and resection of the infundibular stenosis. C, Placement of a fabric patch on the outflow tract of the right ventricle (RV). AO, aorta; PA, pulmonary artery; RA, right atrium. Mortality. For patients with uncomplicated TOF, the mortality rate is 2% to 3% during the first 2 years. Patients at risk are those younger than 3 months and older than 4 years, as well as those with severe hypoplasia of the pulmonary annulus and trunk. 196

Other risk factors include multiple VSDs, large aortopulmonary collateral arteries, and Down syndrome. Complications 1. Bleeding problems may occur during the postoperative period, especially in older polycythemic patients. 2. Pulmonary valve regurgitation may occur, but it is well tolerated. 3. CHF, although usually transient, may require anticongestive measures. 4. Right bundle branch block (RBBB) on the ECG caused by right ventriculotomy, which occurs in over 90% of patients, is well tolerated. 5. Complete heart block (i.e., <1%) and ventricular arrhythmia are both rare. Rastelli Operation Procedure. Figure 14-7 shows a schematic drawing of the procedure. Indications, Timing, and Mortality. Patients with severe hypoplasia or atresia of the right ventricular outflow tract and those with coronary artery anomalies may have the procedure performed at about 5 years of age, at which time adult-sized homograft-valved conduits can be used. The mortality rate for this procedure is 10% or less. Postoperative Follow-up 1. Long-term follow-up with office examinations every 6 to 12 months is recommended, especially for patients with residual VSD shunt, residual obstruction of the right ventricular outflow tract, residual PA obstruction, arrhythmias, or conduction disturbances.

2. Some children develop late arrhythmias, particularly ventricular tachycardia, which may result in sudden death. Arrhythmias are primarily related to persistent RVH as a result of unsatisfactory repair. Complaints of dizziness, syncope, or palpitation may suggest arrhythmias. A 24-hour Holter monitor or exercise test may be needed. 3. Varying levels of activity limitation may be necessary. 4. For patients who have had TOF repair, SBE prophylaxis should be observed throughout life. 5. Children with sinus node dysfunction may require pacemaker therapy. 6. Pacemaker follow-up care is required for patients with implanted pacemakers secondary to surgically induced complete heart block or sinus node dysfunction. Hay Jr., W. W, et al. 2002. Current Pediatric Diagnosis & Treatment. 16th Ed. McGraw-Hill Education Europe. Park, M. K. and George, R. 2002. Pediatric Cardiology for Practitioners. 4th edition. Mosby