|Year : 2014 | Volume
| Issue : 1 | Page : 49-53
Dextrocardia seen in the children in Aminu Kano Teaching Hospital: A report of 8 cases
Aliyu Ibrahim, Asani O Mustafa
Department of Paediatrics, Aminu Kano Teaching Hospital, Bayero University, Kano, Nigeria
|Date of Web Publication||7-Apr-2014|
Department of Paediatrics, Aminu Kano Teaching Hospital, Kano
Source of Support: None, Conflict of Interest: None
Dextrocardia occurs when the heart is located on the right side of the thoracic cavity. It may be classified as dextroposition, isolated dextrocardia, dextrocardia with situs inversus, and dextrocardia with situs ambiguous. Dextrocardia may also be associated with other co-morbidities. We report cases of dextrocardia seen over a seven-year period with varied presentations.
Keywords: Dextrocardia, dextroposition, situs inversus, situs ambiguous
|How to cite this article:|
Ibrahim A, Mustafa AO. Dextrocardia seen in the children in Aminu Kano Teaching Hospital: A report of 8 cases. Nig J Cardiol 2014;11:49-53
| Introduction|| |
Marco Severino, an anatomist surgeon, in 1643 described dextrocardia in situs inversus. It was one of the first recognized congenital heart defect in the medical literature.  Dextrocardia is a rare congenital heart disease characterized by the heart located on the right side of the thorax. Its true incidence is not completely known but it is estimated to occur in about 1 in 12,019 pregnancies in the United States of America;  though several cases of dextrocardia have been documented in Nigeria, some with co-existing cardiac and noncardiac defects, ,,,, its true incidence among Nigerians is unknown. The exact cause of dextrocardia is also unknown, but it has been linked with a number of factors which include autosomal recessive gene inheritance, maternal diabetes, cocaine use and conjoined twinning. , There are four types of dextrocardia that associated with normal positioning of abdominal viscera (situs solitus with dextrocardia), abdominal viscera in reverse position (situs inversus with dextrocardia) or dextrocardia with undifferentiated cardiac chambers (situs ambiguous). The fourth type is a normal heart shifted to the right side of the chest (dextroposition).  In situs solitus with dextrocardia, the heart is commonly associated with congenital cardiac malformations while in dextroposition, the patients often present with symptoms of the extra-cardiac lesion that pushed it to the right hemi-thorax  reference pls. In 1933, Kartagener described the association of sinusitis, bronchiectasis and situs inversus, which was called Kartagener's syndrome.  Though the heart may be structurally normal, it is often associated with respiratory symptoms and infertility in both males and females due to underlying ciliary disorder. However to the best of our knowledge, there is no report of an association of dextrocardia with reactive airway disease. We therefore report cases of dextrocardia with varied co-morbidities seen over a 7-year period from December 2005-2012.
| Case Reports|| |
A five-year-old boy who was initially being followed up in the neurology clinic with delayed developmental milestone and background Down's syndrome. He was referred to the cardiopulmonary clinic for evaluation of associated acyanotic congenital heart disease. He presented to the clinic at 11 th month of life with history of poor weight gain as compared to his peers. No recurrent cough, no breathlessness. A product of full term gestation delivered to a 37-year-old mother. Pregnancy, delivery and neonatal history were not adversely eventful. He was on breastfeeding and it was well tolerated. He was the eighth child of the mother in a polygamous family setting. There was no similar problem amongst his siblings. On examination, the patient was underweight with dysmorphic facie. Cardiovascular examination revealed the dextrocardia with grade two ejection systolic murmur maximal at the left upper sternal margin. Chest X-ray confirmed the diagnosis of dextrocardia. Electrocardiograph (ECG) recorded an inverted P, QRS, T in lead I and positive P, QRS and T waves in lead aVR with progressively decreasing amplitude of the R wave from the right to the left precordial leads. Abdominal ultrasound showed a right sided spleen and a left sided liver. Echocardiography showed secundum type atrial septal defect.
An eight-year-old girl who was presented with a history of recurrent difficulty in breathing of six years duration which is precipitated by exposure to cold wind associated with wheezing. She had several mild episodes over the years often accompanied with cough which were mostly nocturnal. There was no associated dark discoloration of mucous membrane, and no history of body swelling. Her father was a known asthmatic. The patient had received medications at a general hospital with significant improvement. However, she had two severe episodes on two occasions which necessitated hospitalization. She was the fifth child of a father of eight children in a polygamous non-consanguineous family setting. She had an elder sibling who was an asthmatic. General physical examination revealed no significant findings. However, cardiovascular system examination revealed cardiac apex situated at the fifth right intercostal space mid clavicular line with normal first and second heart sounds only. Examinations of other systems revealed no abnormality. Chest X-ray and echocardiography done confirmed the diagnosis of dextrocardia [Figure 1]; no other structural defect was detected. She had a normal abdominal ultrasound study. She had pulmonary function test which was in keeping with an obstructive airway (respiratory) disease; she was diagnosed with isolated dextrocardia and childhood asthma. She responded to bronchodilator therapy and had ever remained stable with normal growth and development.
|Figure 1: Subcostal view showing the inferior vena-cava entering a leftward right atrium and chest X-ray showing dextrocardia (case 2)|
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An eighteen-month-old first twin who was presented at the seventh month of life with complaints of recurrent difficulty in breathing since birth and poor weight gain. Difficulty in breathing was associated with coughing. There was associated history of poor suck and excessive sweating during feeds. There was no dark discoloration of lips or mucous membrane. The patient was a product of full term twin gestation. He was the first of a set of twins. The second twin was still birth. On examination, the cardiovascular system examination revealed a right sided cardiac pulsation. The first and second heart sounds with a pansystolic murmur grade 3/6 at the lower right sternal edge were present. All other systems were normal.
Chest X-ray showed a dextrocardia with cardiomegaly while echocardiography revealed a partial atrioventricular canal defect. He had a normal abdominal ultrasound study. Since diagnosis patient has being followed up in the cardiology clinic. He is on spironolactone and frusemide and has remained relatively stable awaiting corrective cardiac surgery.
The patient was a five-year-old girl who first presented to at the age of four years with complaints of recurrent cough, breathlessness and fever since birth. There was no bluish discoloration of lips and mucous membrane but had difficulty breathing especially while sucking from the breast. There was associated poor weight gain. Pregnancy, delivery, and neonatal periods were normal. She was the second child of the mother who had two other children in a polygamous family setting. She had a younger sibling who was also later diagnosed with dextrocardia. She was a product of consanguineous marriage. Parents are first cousins. On examination, she was small for age. Significant finding was in the cardiovascular system; the cardiac pulsation and heart sounds were detected on the right side of the chest. A chest X-ray revealed a right sided cardiac shadow [Figure 2]. ECG recorded an inverted P, QRS, T in lead I and positive P, QRS and T waves in lead aVR with progressively decreasing amplitude of the R wave from the right to left precordial leads. Abdominal ultrasound showed a right sided spleen and a left sided liver. Echocardiography showed a dextrocardia with partial atrioventricular canal defect. A diagnosis of situs inversus with AV canal defect was made. She was placed on oral frusemide and spiranolactone and has remained stable. The patient has being followed up in the cardiology clinic while awaiting corrective heart surgery.
A three-year-old boy who was brought to the hospital by the parents at the third day of life for check up and evaluation on account of having an older sibling with dextrocardia. The child had been doing well with no cardiopulmonary symptoms. He sucked well from the breast. He was delivered at term, pregnancy, delivery was not adversely eventful and the nutritional history was normal. He was the third child of both parents. The second was a female and is being followed up in the cardiology clinic of the hospital for dextrocardia. First sibling had been evaluated and was normal. He was a product of consanguineous marriage with parents being first cousins. Both parents are normal. On examination, his cardiac apex was located at the fourth right intercostal space with normal first and second heart sounds. A chest X-ray showed the cardiac shadow located at the right side of the thorax with the apex pointing rightward [Figure 3]. ECG recorded an inverted P, QRS, T in lead I and positive P, QRS and T waves in lead aVR with progressively decreasing amplitude of the R wave from the right to left precordial leads. Abdominal ultrasound showed a right sided spleen and a left sided liver. Echocardiography also confirmed the diagnosis of dextrocardia with no other morphologic abnormality. A diagnosis of situs inversus totalis (dextrocardia with situs inversus) was made.
|Figure 3: Chest X-ray dextrocardia with gastric gas bubble on the right, while the liver is on the left side (case 5)|
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A two-month-old infant was presented to the emergency pediatric unit with three day history of fever, cough and difficulty in breathing. Fever was high grade intermittent, cough was non-paroxysmal, no known aggravating or relieving factors had associated to history of difficulty in breathing which was noticed to be worsening thus, preventing child from sucking well, that was the first episode of difficulty in breathing. There was no history of force feeding or choking while feeding and no cyanosis. On examination, he was acutely ill looking in respiratory distress with exfoliated skin. He was febrile with axillary T˚ 38˚C, he was pale, not dehydrated, no significant peripheral lymph node enlargement. He was tachypneic with respiratory rate of 80 breaths/min, oxygen saturation in room air was 89% (hypoxemic), and he had reduced breath sounds and bilateral basal crepitations posteriorly. He was tachycardiac with pulse rate of 170 beat per minute, the apex beat was palpated on the right fifth intercostal space at the anterior axillary line, the heart sounds were loudest on the right with 1 st , 2 nd and 3 rd heart sounds, no murmur. Abdominal examination revealed a tender hepatomegaly (6 cm below the right coastal margin.) located on the right hypochondrium. Full blood count showed hemoglobin concentration of 7.9 g/dl, (HCT 21.1%). Total white cell count was 7.7 × 10 9 /l with relative neutrophilia. Serum urea and electrolytes were essentially normal. Retroviral screening of mother, father and child were reactive by determine/unigold. Blood culture yielded proteus specie sensitive genticin, ceftriaxone, and co-trimoxazole. Chest radiograph revealed bilateral patchy opacities with the heart on the right side. The abdominal ultrasound revealed right sided liver and the spleen on the left side. He had echocardiography which revealed he has only dextrocardia. Diagnosis of severe bronchopneumonia in heart failure with isolated dextrocardia in a retroviral disease exposed infant. He had oxygen, frusemide, blood transfusion, antibiotics following which he improved remarkably.
A seven-month-old girl presented with complaint of recurrent difficulty in breathing associated with fever and history of poor sucking since the age of four months. There was poor weight gain noticed since onset of illness; there was no history of bluish discoloration of the lips or the mucous membrane. She was a product of term pregnancy in a non-consanguineous marriage. She was small for age, was not cyanosed and had no digital clubbing. She had ankyloglossia. She was tachycardiac and tachypneic with her apex beat maximal on the right fifth intercostal space at the anterior axillary line. The liver was enlarged and palpated at the right hypochondrium. Her chest X-ray revealed dextrocardia [Figure 4] her hemoglobin concentration was 12.5 g/dl, the ECG showed an inverted P, negative QRS, T in lead I, and upright P, QRS, T in aVR and progressive decreasing QRS complexes from the right to the left precordial leads. Echocardiography also revealed a secundum type atrial septal defect and a perimembranous ventricular septal defect.
|Figure 4: Isolated dextrocardia, the liver shadow showing on the right side (case 7)|
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An eight-year-old girl presented with complaint of recurrent wheezing with associated cough mostly nocturnal and recurrent difficulty with breathing since the age of six years. There was no history of weight loss, no history of halitosis, hemoptysis, darkened lips or extremities. She was of a non-consanguineous marriage, and no similar problem in any of the other family members. On examination, there were no significant pulmonary findings, but her cardiac apex beat was located at the right side, chest X-ray showed the heart in the right side and the lung fields were normal while an abdominal ultrasound showed the liver on the left and the spleen was rightward, she had a pulmonary function test which reported an obstructive airway disease pattern. A diagnosis of situs inversus totalis with and childhood asthma, she responded to bronchodilators therapy and had ever remained stable with normal growth and development.
| Discussion|| |
Dextrocardia could occur isolated with situs solitus or associated with complete reversal of location of the body organs called situs inversus totalis. In isolated dextrocardia the heart apex points rightward but the atria relationship are properly maintained in their location but the ventricles are rotated along their longitudinal axes therefore the left ventricle lies predominantly anterior, while the right ventricle forms the right border of the heart; this has been known by several names like dextro-version, dextro-rotation. The risk of congenital heart disease is increased in the patients with isolated dextrocardia, often seen in about 90-95% patients affected compared to 0-10% of the patients with dextrocardia and situs inversus totalis; three out of the five cases (Cases 1, 3 and 7) with dextro-version had associated structural heart defect compared to only one out of the three with situs-inversus-totalis (Case 4). Common defects often seen are ventricular septal defect, atrial septal defect, pulmonary stenosis and transposition of the great arteries. In dextro-version, since the right-left atrial orientation is preserved, the ECG P-wave in the limb leads I and aVL are upright, while they are usually inverted in dextrocardia with situs-inversus-totalis; however, cases with mirror-image dextrocardia with upright P-waves in lead I, as well as case of dextro-version with negative P-waves in lead I,  have been reported. However, cases 1, 2, 3 and 7 had situs solitus but the orientation of the cardiac situs was that of a mirror image type dextrocardia as was confirmed by the ECG and echocardiography; why this occurs is not know but it is similar to that reported by Campbell et al. who documented similar findings,  but cases 2 and 6 had no other intracardiac abnormality which may be attributed to, they being of the mirror image type morphology because a true dextroversion has an almost 95% chance of an associated intracardiac defect. Situs inversus totalis may have an associated complaint of recurrent sinus and chest infection with bronchiectasis known as kartagener syndrome.  Overtime they may present with failure to thrive. Our patients (Cases 2 and 8) however presented with nocturnal coughing and wheezing which characteristically responded to bronchodilators and there were no complaints of chronic sinus or chest infection as often seen in the patients with primary ciliary dyskinesia, furthermore their chest X-rays had no features suggestive of bronchiectasis. The relationship between asthma and dextrocardia is however not clear and has not been reported before now to the best of our knowledge; this could have been co-morbidity since the first case had family history of asthma in a first degree relative. The first case was also diagnosed with Down's syndrome; common cardiac defect seen in Down's syndrome include atrioventricular septal defect, ventricular septal defect, atrial septal defect, tetralogy of Fallot, isolated persistent patency of the arterial duct and others in 43%, 32%, 10%, 4%, and 5% respectively;  but our patient had an isolated dextrocardia, which had not been reported in the patients with Down's syndrome before now. Therefore, this may expand the phenotypic spectrum of diseases seen in the patients with Down's syndrome. The third case was a surviving member of a set of identical twin; he had ECG and echocardiographic features of dextro-version with multiple intracardiac defects and situs solitus. Although mirror image dextrocardia and situs inversus are often seen in the mirror image identical twinning, conjoined twinning, and our patient rather had dextro-version similar to that reported by Joyce  and Aired,  though genetic predisposition has been implicated, the exact gene and mechanism involved is unclear.
| Conclusion|| |
Though dextrocardia has been classified broadly into mirror image dextrocardia, dextro-version and secondary dextrocardia; mirror image dextrocardia could still occur in the patients with normal abdominal organ situs, furthermore, there is the need for clinicians to watch out for other co-morbid diseases which may not be commonly associated with dextrocardia which may further expand the knowledge of this rare cardiac disease.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4]