Nigerian Journal of Cardiology

CASE REPORT
Year
: 2019  |  Volume : 16  |  Issue : 2  |  Page : 107--110

Dextrocardia with situs inversus in an adult Nigerian


Bonny Adah Ejeh, Yekeen Ayodele Ayoola, Henry Ifeanyichukwu Okolie, Solomon Sule Danbauchi 
 Department of Medicine, Federal Teaching Hospital, Gombe, Nigeria

Correspondence Address:
Dr. Yekeen Ayodele Ayoola
Department of Medicine, Federal Teaching Hospital, Gombe
Nigeria

Abstract

Situs inversus with dextrocardia refers to complete inversion of the position of the thoracic and abdominal viscera. Dextrocardia occurs when the heart is located in the right side of the thoracic cavity with its base-to-apex axis directed to the right and inferiorly. It may be isolated or associated with cardiac or alimentary malformations. Our patient had dextrocardia with situs inversus. The patient is a 32-year-old woman that presented with recurrent respiratory symptoms but no cardiac symptoms. Dextrocardia with situs inversus was confirmed by chest radiograph, electrocardiography, and abdominal ultrasound. Echocardiography established atrioventricular great arteries and ventricular concordance with no valvular abnormalities.



How to cite this article:
Ejeh BA, Ayoola YA, Okolie HI, Danbauchi SS. Dextrocardia with situs inversus in an adult Nigerian.Nig J Cardiol 2019;16:107-110


How to cite this URL:
Ejeh BA, Ayoola YA, Okolie HI, Danbauchi SS. Dextrocardia with situs inversus in an adult Nigerian. Nig J Cardiol [serial online] 2019 [cited 2020 Jun 3 ];16:107-110
Available from: http://www.nigjcardiol.org/text.asp?2019/16/2/107/270684


Full Text



 Introduction



Dextrocardia is an abnormal congenital positioning of the heart where its base-to-apex axis directed to the right and caudad. Situs solitus is the normal position, and situs inversus is the mirror image of situs solitus. Situs inversus with dextrocardia is also called situs inversus totalis because the cardiac position, as well as the atrial chambers and abnormal visceral is the mirror image of the normal anatomy.

Dextrocardia with complete situs inversus occurs in 2/10,000 live births with the incidence of congenital heart disease among them as low as 3%.[1] Dextrocardia with situs ambiguous is less common and occurs in 1/20,000 live births.[1] The incidence of congenital heart disease very high in patients with situs ambiguous. It could be as high as 90%.[2] About 50% of patients with dextrocardia and congenital heart disease have situs solitus and situs ambiguous accounting for the remaining.[3]

Dextrocardia with situs inversus is usually an incidental finding. The recognition of this cardiac malposition is based on physical signs, electrocardiography, and chest radiography.[4]

 Case Report



Mrs. M. M, a 32-year-old woman, presented to the medical emergency unit of Federal Teaching Hospital, Gombe, with a 2-week history of high-grade fever, 1-week history of cough productive of mucoid, foul-smelling sputum associated with pleuritic chest pain, exertional dyspnea, and nasal discharge.

There was a history of recurrent symptoms of cough, dyspnea, and nasal discharge dating back to childhood with a frequency of about ten times per year but has reduced to 3–5 times per year in the past 5 years.

Her presenting symptoms were recorded as the third episode in the past 6 months. She is a known asthmatic but denies any history of hypertension or diabetes.

She is a divorcee who was married in a monogamous setting to her 49-year-old cousin for 10 years with a history of primary infertility. She has a regular menstrual cycle and was evaluated for infertility with no structural or functional abnormality detected. Her ex-husband had seminal fluid analysis done that revealed some abnormalities and he declined further evaluation. Her other siblings are married with children.

On examination at presentation, she was febrile (T°C-39), not pale, and anicteric with no digital clubbing. Examination of the cardiovascular system revealed a pulse rate of 108 beats/min and her blood pressure was 110/70 mmHg. The apex beat was not found on the left side but was found to be at the right fifth intercostal space, midclavicular line. The heart sounds were normal intensity of the first and second heart sounds only.

She was tachypneic with coarse crepitations in the left middle and lower lung zones and the right lower lung zone. The abdominal examination revealed no organomegaly, and liver dullness was detected in the left hypochondrium with normal liver span and tympanic notes over the right hypochondrium.

An initial impression of community-acquired pneumonia with situs inversus and suspicion of Kartagener syndrome was made.

Chest radiograph – posterior-anterior view [Figure 1] showed the heart in the right hemithorax with the base-to-apex axis pointing toward the right, the aortic arch was located on the right, and the lung fields were clear. The thoracic cage was normal, and the gastric bubbles were located on the right.{Figure 1}

An electrocardiogram (ECG) [Figure 2] showed a heart rate of 78 beats/min, normal sinus rhythm, inversion of P-waves in lead 1 and aVL, dominant S-waves in lead 1, V1 to V6, reversed R-wave progression in chest leads, low-voltage QRS in V4 to V6, and inverted T-waves in lead 1 and aVL.{Figure 2}

Echocardiography showed normal great arteries concordance with the ventricles, atrioventricular concordance, and normal atrioventricular valves.

Abdominal scan showed a normal liver and gall bladder located in the left hypochondrium while the spleen was located in the right hypochondrium. Both kidneys were normal in position, size, and outline [Figure 3] and [Figure 4]. An assessment of situs inversus was made ultrasonographically.{Figure 3}{Figure 4}

The final diagnosis of dextrocardia with situs inversus to exclude Kartagener syndrome was made. Sputum microscopy culture and sensitivity, sputum GeneXpert, and chest computed tomography (CT) scan were requested. She was placed on a combination of cefuroxime and azithromycin pending the results of sputum test. Her sputum microscopy, culture, and sensitivity yielded growth of Klebsiella pneumoniae, and full blood count showed a PCV of 42% with neutrophil leukocytosis with toxic granulation. Following the completion of antibiotics, she became symptom free.

 Discussion



Dextrocardia is one of the cardiac anomalies that present with unusual and specific electrocardiographic findings which include inversion of P-waves in lead 1 and aVL, dominant S-waves in lead 1 and V1 to V6, reversed R-wave progression in chest leads, low-voltage QRS in V4 to V6, flattened T-waves in V4 to V6 and aVR, and inverted T-waves in lead 1 and aVL. These findings were present in our patient and were consistent with the ECG findings in dextrocardia in other studies.[5],[6]

The chest X-ray of our patient [Figure 1] demonstrated the mirror image nature of dextrocardia. Danbauchi and Alhassan in 2002 reported two cases of dextrocardia with situs inversus. The first patient was a 35-year-old man who presented for the first time with respiratory symptoms but no cardiac symptoms, and the second patient, a 14-year-old male presented with cardiac symptoms.[6]

Nawaz et al. reported a case of two newborns with situs inversus associated with congenital duodenal obstruction.[7]

According to a retrospective chart review at the Children's and Women's Health Centre of British Columbia in Vancouver, Canada, based on a population-based study of cardiac malformation, an estimated incidence of dextrocardia has been reported to be 1 in 12,019 pregnancies.[8]

Dextrocardia with situs inversus may occur with other features in the respiratory tract. Kartagener syndrome occurs in about 25% of individuals with dextrocardia. Kartagener syndrome is characterized by dextrocardia with situs inversus, sinusitis, and bronchiectasis. It is often associated with primary ciliary dyskinesia.[9] Our patient presented with respiratory symptoms, and Kartagener syndrome was suspected. The chest X-ray findings were unremarkable for Kartagener syndrome, and the diagnosis was not confirmed because a chest CT scan was not done due to financial constraints.

The genetic basis for dextrocardia is not well understood. Familial tendency to dextrocardia was described by Ibrahim in 2013 in Ibadan.[10] The type of dextrocardia associated with respiratory abnormalities was said to be autosomal recessive. Both autosomal recessive and X-linked variants of dextrocardia have been described.[11]

 Conclusion



A well-interpreted ECG is a useful tool in the diagnosis of dextrocardia with situs inversus. Hence, analysis of this relatively simple and noninvasive diagnostic tool allows for a suspicion of a cardiovascular anomaly in a resource-limited setting like ours.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

References

1Ellis H. Clinical Anatomy: Applied Anatomy for Students and Junior Doctors. 9th ed. United Kingdom: Wiley-Blackwell; 1997. p. 45.
2Umberto S, Donald GR, Owing WK. Dextrocardia: Angiographic study and classification. Am J Cardiol 1973;32:965-72.
3Fuster V, Walsh RA, Harrington RA. Hurst's the Heart. 13th ed. United States: McGraw-Hill Companies, Inc.; 2011. p. 1867-9.
4Stanger P, Rudolph AM, Edwards JE. Cardiac malpositions. An overview based on study of sixty-five necropsy specimens. Circulation 1977;56:159-72.
5Uchenna DI, Jesuorobo DE, Anyalechi JL. Dextrocardia with situs inversus totalis in an adult Nigerian: A case report. Am J Med Sci 2012;2:59-61.
6Danbauchi SS, Alhassan MA. Case report: Dextrocardia with situs inversus; two cases presenting differently. Niger Postgrad Med J 2002;9:248-52.
7Nawaz A, Matta H, Hamchou M, Jacobez A, Trad O, Al Salem AH. Situs inversus abdominus in association with congenital duodenal obstruction: A report of two cases and review of the literature. Pediatr Surg Int 2005;21:589-92.
8Bohun CM, Potts JE, Casey BM, Sandor GG. A population-based study of cardiac malformations and outcomes associated with dextrocardia. Am J Cardiol 2007;100:305-9.
9Maldjian PD, Saric M. Approach to dextrocardia in adults: Review. AJR Am J Roentgenol 2007;188:S39-49.
10Ibrahim A. Familial dextrocardia in a Nigerian family. Niger Med J 2013;54:282-3.
11al-Khadra AS. Images in cardiovascular medicine. Mirror-image dextrocardia with situs inversus. Circulation 1995;91:1602-3.