|Year : 2019 | Volume
| Issue : 3 | Page : 149-152
Management of tetralogy of Fallot with Blalock–Taussig shunt alone in a low-resource setting
Ehi Judith Ogbemudia1, Stanley Okugbo2
1 Department of Medicine, University of Benin Teaching Hospital, Benin City, Edo State, Nigeria
2 Department of Surgery, University of Benin Teaching Hospital, Benin City, Edo State, Nigeria
|Date of Submission||27-Apr-2018|
|Date of Acceptance||21-Aug-2018|
|Date of Web Publication||26-Sep-2019|
Dr. Ehi Judith Ogbemudia
Department of Medicine, University of Benin Teaching Hospital, Benin City, Edo State
The outcomes of management of Tetralogy of Fallot (TOF) in children with only BT shunts have not been widely reported. Therefore, we present a 29-year-old man with complaints of progressive easy fatigability and effort intolerance. He was diagnosed with TOF in infancy and had both left- and right-sided BT shunts without corrective surgery. Examination revealed an asthenic young man with conjunctival plethora, cyanosis, digital clubbing, and hypertension. Chest X-ray, electrocardiography, and echocardiography revealed the typical anomalies of TOF. He has been referred for corrective surgery. Despite two previous BT shunts, the investigations still revealed the structural anomalies of TOF; this confirms BT shunt is not the definitive treatment but a palliative measure. Total corrective surgery remains the definitive treatment of TOF. Patients and their caregivers should be counseled on this and on the need for early corrective surgery.
Keywords: Blalock, Fallot, shunt, Taussig, tetralogy
|How to cite this article:|
Ogbemudia EJ, Okugbo S. Management of tetralogy of Fallot with Blalock–Taussig shunt alone in a low-resource setting. Sahel Med J 2019;22:149-52
|How to cite this URL:|
Ogbemudia EJ, Okugbo S. Management of tetralogy of Fallot with Blalock–Taussig shunt alone in a low-resource setting. Sahel Med J [serial online] 2019 [cited 2019 Oct 13];22:149-52. Available from: http://www.smjonline.org/text.asp?2019/22/3/149/267894
| Introduction|| |
Tetralogy of Fallot (TOF) is a cyanotic congenital heart disease. It is composed of ventricular septal defect (VSD), overriding of the aorta, right ventricular outflow tract obstruction, and right ventricular hypertrophy. TOF is the most common cyanotic congenital heart disease, and it represents 10%–26.2% of all congenital heart diseases.,,
The definitive management of TOF is total corrective surgery, but palliation is achieved with Blalock–Taussig (BT) shunt which is a surgically created anastomosis between the subclavian or carotid artery and pulmonary artery. The aim is to enhance the growth of pulmonary arteries by increasing pulmonary perfusion. It also alleviates cyanosis and improves exercise tolerance. BT shunt was the first method of treatment before corrective surgery was developed. At present, it is used as a bridge for total correction in patients who cannot have a primary correction.
However, in low-resource settings, many patients with BT shunt inadvertently take it as definitive therapy because of unavailability of open-heart surgery and high costs of cardiac care. Cases of children managed with only BT shunts and their outcomes in low-resource settings have not been widely reported.
Therefore, we present a 29-year-old man who was diagnosed with TOF in childhood and had both left and right BT shunts at different times but without corrective surgery.
| Case Report|| |
Approval was obtained from the research and ethics committee, and patient gave informed consent. Mr. BC, a 29-year-old graduate, was referred to the cardiac clinic on account of progressive easy fatigability and dyspnea on exertion of 3 years' duration. He did not have orthopnea, paroxysmal nocturnal dyspnea, or cough.
He was diagnosed with TOF in infancy for which he had left-sided BT shunt at 4 years of age. He had a second BT shunt on the right side at age 19 when symptoms recurred, but he never had corrective surgery because he claimed that he was not informed about it. He was recently diagnosed to have hypertension and he is being managed for polycythemia by the hematologist. He is the third child in a monogamous setting of seven children. None of his parents or siblings has similar complaints.
On examination, he had normal facies and was asthenic with a body mass index of 18.4 kg/m2. There was conjunctival plethora, central and peripheral cyanosis, Grade 4 digital clubbing, and mild bilateral pitting edema up to the lower third of his legs.
The pulse rate was 98 beats/min, regular, and of normal volume. The arterial wall was thickened and there was locomotor brachialis. His blood pressure was 130/100 mmHg, and the jugular venous pressure was elevated to 5 cm above the sternal angle. The apex beat was in the 5th left intercostal space lateral to the midclavicular line, and there was left parasternal heave. The 1st and 2nd heart sounds were present with an ejection systolic murmur in the pulmonary area. There was no bruit over the shunt.
He has bilateral posterolateral thoracotomy scars. The respiratory rate was 16 breaths/min, and the lungs were clear clinically. The abdomen was flat and soft with no areas of tenderness. The liver was enlarged 4 cm below the right costal margin, but the spleen and kidneys were not palpable. Renal function test was essentially normal, but his packed cell volume was 64%.
Chest X-ray [Figure 1] revealed a boot-shaped heart, mild cardiomegaly (cardiothoracic ratio of 53.5%), and right-sided aortic arch. Electrocardiography [Figure 2] showed sinus rhythm with right ventricular hypertrophy, right-axis deviation, and incomplete right bundle branch block.
|Figure 1: Chest X-ray posteroanterior view. Boot-shaped heart, cardiomegaly, right-sided aortic knuckle|
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|Figure 2: Electrocardiography. Right-axis deviation incomplete right bundle branch block|
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Echocardiography [Figure 3] revealed a subaortic VSD, aortic override, and right ventricular hypertrophy. [Figure 4] showed infundibular stenosis. There was left ventricular hypertrophy with impaired systolic function.
|Figure 3: Echocardiography (parasternal long-axis view) VSD = Ventricular septal defect, OA = Overriding of aorta|
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|Figure 4: Echocardiography (parasternal short-axis view) RVOTO = Right ventricular outflow tract obstruction|
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He was placed on heart failure therapy and has been referred to a cardiothoracic center for total correction.
| Discussion|| |
Our patient had a left-sided BT shunt when he was 4 years old. This was appropriate because BT shunt was the first-line treatment at that time. However, in contemporary times, the trend is to perform primary correction often electively in the 1st year of life. BT shunt is now reserved for those with contraindications to primary correction such as very small pulmonary arteries, low birth weight, and multiple anomalies.
Symptoms recurred years later, necessitating the insertion of a second BT shunt on the right side at age 19. This is not surprising as BT shunts are known to become inadequate as the child grows, confirming the need for total correction. However, corrective surgery was not available, so he had to have another shunt. Many patients can only afford the palliative procedures and may not have access to more definitive correction.
He has survived into adulthood without corrective surgery, although he presently has polycythemia, hypertension, and heart failure. The development of these complications is not surprising; BT shunt does not stop the progression of the disease as the lesions still persist. However, the shunts must have contributed to his longevity. Other favorable factors include hypertension, left ventricular hypertrophy, and left-to-right shunt. However, these patients are still at risk of life-threatening arrhythmias and sudden cardiac event. Thus, total correction is still indicated.
For adults with TOF, total correction is routinely performed as it provides an opportunity for improved prognosis and long-term survival. Multiple series of older patients undergoing total correction have shown good outcomes comparable to those on younger patients. It is important to ensure that patients are properly counseled on the need for total correction. The need for cardiac care does not end with total correction though. There is still a higher than normal incidence of cardiac events and challenges with exercise and physical activities. Thus, expectations and help must be clearly spelt out.
In conclusion, despite two previous BT shunts, the investigations still revealed the structural anomalies of TOF; this confirms that BT shunt is not the definitive treatment but a palliative measure. Total corrective surgery remains the definitive treatment of TOF. Patients and their caregivers should be informed about this and on the need for early total corrective surgery.
Ethical approval and declaration of patient consent
Ethical approval protocol number ADM/E 22/A/ VOL. VII/14630 from University of Benin teaching hospital (UBTH) was obtained on April 20th 2018.
The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given his consent for his images and other clinical information to be reported in the journal. The patient understands that name and initial will not be published and due efforts will be made to conceal identity.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4]