GULU UNIVERSITY MEDICAL JOURNAL

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Gulu University Medical Journal (GUMJ) 2009/2010 Vol 5. 1. A diagnosis is most likely not possible at this stage because history is not adequate. Though she may have a few rough ideas in mind. Possible impressions could be; • Pneumonia in view of the cough associated with difficulty in breastfeeding. • Febrile illness in view of the fever for example malaria and bacterial meningitis. • Anaemia in view of difficulty in breathing. 2. At this stage she has only been able to ascertain the presenting complaint, history of presenting complaint, review of other systems. So she has to inquire more about other aspects of the history and examine the patient to be sure of the diagnosis. 3. Right from the beginning as Dr Doreen gets engraved into a conversation with the patient’s mother, you have to keep in mind important aspects like DIB * 4/7 and cough *3/7. It’s important to note that the DIB is not worsened on lying down and no h/o body swelling. As you proceed, it’s wise to ask yourself a few questions like: why is the child always in and out of health facilities? , what does the ‘hole in the heart” imply? Other aspects are that she delivered from home; the child’s birth was preterm and she was under weight. She was breastfed less than the recommended 6 months, and does not feed adequately. 4. From the history and examination results, the child most likely has a congenital heart disease (CHD). 5. The diagnosis is Ventricular Septal Defect (VSD). Congenital means existing before or at birth. Congenital heart diseases can be classified according to whether they cause cyanosis or characterized by presence of a shunt. They can present with a heart murmur (which Lamunu had), heart failure or cyanosis. Examination revealed no cyanosis. Hence the condition is acyanotic. The shunt can be at the atrial wall or the ventricular wall level. Lamunu has ventricular septal defect with a left to right shunt. Defects of the ventricular septum are the commonest and usually occur as isolated defects and as one component Quiz Answers of a combination of anomalies. The opening is usually single and situated in the membranous portion of the septum i.e. perimembranous (75%). Malalignment defects may due to malalignment of infundibular septum and trabecular muscular septum; muscular defects may be located anywhere in the muscular septum (5-20%). Presentation depends primarily on defect size and status of the pulmonary vascular bed, rather than on the location of the defect. Shunting depends on the relative pulmonary and systemic vascular resistance. In large defects there is equilibration of right and left ventricular pressures. A large VSD with a low pulmonary resistance, degree of left to right shunt is increased. Usually large defects come to medical and, often, surgical attention very early in life. Natural history of ventricular septal defect ranges from spontaneous closure to congestive cardiac failure (CCF) and death in early infancy. The patient’s defect is of moderate size and spontaneous closure has not taken place and it could be that she has developed in view of the dyspnea, little interest in playing and difficulty in B/F. Among others are the possible development of pulmonary vascular obstruction (more with large) defects, Right Ventricular (RV) outflow tract obstruction, aortic regurgitation, and infective endocarditis. Lamunu’s DIB and cough indicate pulmonary hypertension though no haemoptysis as yet, though no worry for severe pulmonary vascular obstruction (Eisenmenger Syndrome) which usually presents with symptoms in adult life including exertional dyspnea, chest pain, syncope, and haemoptysis. Thus, the importance of correcting moderate-large defects surgically early in life when pulmonary vascular disease is still reversible or not yet developed. The right-to-left shunt leads to cyanosis, clubbing, and erythrocytosis. In all patients, the degree to which pulmonary vascular resistance is elevated before operation is a critical factor determining prognosis. If the pulmonary vascular resistance is one-third or less of the systemic value, progression of pulmonary vascular disease after operation is unusual. However, for a moderate to severe increase in pulmonary vascular resistance preoperatively, either no change or a progression of pulmonary vascular disease is common Gulu University Medical Students’ Association (GUMSA) Passion for life 64
Gulu University Medical Journal (GUMJ) 2009/2010 Vol 5. postoperatively. RV outflow tract obstruction usually develops in 5 to 10% of patients who present in infancy with a moderate to large left-to-right shunt. With time, as subvalvular RV outflow tract obstruction progresses, findings in the patients begin to resemble more closely those of the cyanotic tetralogy of Fallot. 5% of patients develop incompetence of the aortic valve due to insufficient cusp tissue or prolapse of the cusp through the inter ventricular defect; the aortic regurgitation then complicates and usually dominates the clinical course. 6. Management depends mainly on the child presents and presence of complications. With a low SaO2, the child needs oxygen therapy. To control CCF you need to limit her physical activity and reducing salt intake but increase caloric intake. She needs to be started on an inotropic like digoxin and a loop diuretic furosemide 1mg/kg. In this child it’s evident that the defect is persistent and surgery is likely option. Surgery is not recommended for patients with normal pulmonary arterial pressures with small shunts (pulmonary-to-systemic flow ratios of 1.5 to 2.0:1.0). Operative correction is indicated when there is a moderate to large left-toright shunt with a pulmonary-to-systemic flow ratio of 1.5:1.0 or 2.0:1.0, in the absence of prohibitively high levels of pulmonary vascular resistance. The patient has mild malnutrition due to reduced intake. In such a case correcting the primary cause is baseline for management. In addition the parents have to be counseled and educated about the child’s condition. This prepares them for any outcome. Natural course depends on size of defect. 30%- 50% small defects close spontaneously in first 2years of life. Observation and prophylaxis for indicated procedures are important in this case. 7. Complications • Left ventricular volume overload • Pulmonary over circulation • Compromise of systemic • Endoocarditis 8. Differentials • Mitral or tricuspid regurgitation • Patent ductus arteriosus • Atrial septal defects • AV canal defects Gulu University Medical Students’ Association (GUMSA) Passion for life 65
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