Dextrocardia and isolated laevocardia. II. Situs inversus and isolated dextrocardia.
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Adolescent↗
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[Dextrocardia--mirror image dextrocardia and dextroversion].
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Adult↗
[Therapeutic case of multiple heart anomalies with dextrocardia--situs inversus complicated by dextrocardia, partial abnormal return of the pulmonary vein, atrial septal defects and defect of the inferior vena cava].
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Abnormalities, Multiple↗
Dextrocardia: an analysis of cardiac structures in 125 patients.
BACKGROUND: Dextrocardia is associated with multiple and complex congenital cardiac anomalies. Precise anatomical diagnosis is essential for successful surgery. Spectrum of congenital malformations in cases of dextrocardia is based primarily on two-dimensional echocardiographic studies. The purpose of the current study was to use colour Doppler echocardiography in large number of patients. METHODS: Patients of dextrocardia were studied retrospectively, by reviewing database of our echocardiographic laboratory over last 10 years. Standard criteria for diagnosis of situs were used. Detailed segmental analysis for cardiac anatomy and associated malformations was done using previously suggested and well accepted terms and definitions. Cardiac anatomy was confirmed on catheterization and during surgery in few cases. RESULTS: Of total 125 patients, dextrocardia was most common with situs inversus (39.2%) followed by situs solitus (34.4%) and situs ambiguous [26.4% (right isomerism in 18.4% and left isomerism in 8.0%)]. Mean age was 9.2+/-11.2 years (range; 3 days to 60 years), 82 males and 43 females. In situs inversus dextrocardia, majority (73.4%) had concordant atrioventricular (AV) connections while discordant AV connections and univentricular atrioventricular connections (UVAVC) were present in 12.2 and 14.3% patients, respectively. Majority of patients with concordant AV connections (72.2%) also had concordant VA (ventriculo-arterial) connections (conotruncal anomalies were commonest). Similarly, majority of patients with discordant AV connections (66.7%) also had discordant VA connections. Commonly (44.9%), these patients presented with decreased pulmonary blood flow (Qp). Total 28.6% patients had normal intracardiac anatomy (10.2% presented with rheumatic heart disease). In situs solitus dextrocardia, majority (51.2%) had AV concordance while discordant AV connections and UVAVC were present in 41.9 and 7.0% patients, respectively. In patients with concordant AV connections, majority (77.2%) had VA concordance (majority presented with increased Qp due to pre or post-tricuspid shunts). Similarly, majority of patients with discordant AV connections (88.9%) also had discordant VA connections (88.9% presented with decreased Qp). Only 7.0% patients with situs solitus dextrocardia had normal intracardiac anatomy. The striking features of right isomerism were male predominance (male:female ratio 2.2:1), cyanosis with decreased Qp in 86.9%, and high incidence of UVAVC and venous system anomalies (39.1% each). Striking features of left isomerism were biventricular ambiguous AV connections in all except one, presentation with increased Qp in 60.0% and presence of inferior vena caval interruption in 60.0% patients. CONCLUSIONS: Present study, largest study of dextrocardia till date reconfirms that these patients have variable intracardiac anatomy depending upon their situs and types of segmental connections. These patients can present with different haemodynamic subsets, which can be correctly identified by colour Doppler echocardiography. Diagnostic accuracy and a better understanding of the various types of dextrocardia are essential, since improved surgical techniques have made it possible to correct many of these complex abnormalities.
Adolescent↗
Diagnosis and surgical treatment of congenital dextrocardia.
OBJECTIVE: To understand the pathological features of congenital dextrocardia and explore its diagnosis and surgical treatment. METHOD: The clinical data from 32 cases of congenital dextrocardia were collected to understand the major cardiac anomalies and evaluate the diagnostic approaches of this disease. Analysis of the effect of surgical treatment was also conducted in 16 among the 32 cases. RESULTS: Complex anomalies such as transposition of great artery (TGA), single ventricle (SV), common trunk (CMT) formation for the arteries were found in 8 out of 13 mirror-image dextrocardia cases, most of the dextroversion (DV) accompanied by physiological correction of the TGA (C-TGA, 11/17) which often involved ventricle septal defect (VSD) and pulmonary stenosis (PS, 8/11). Another 2 cases diagnosed as isolated dextrocardia (IDC) also suffered TGA combined with complete atrioventricular canal (CAVC) defect. Definite diagnoses of dextrocardia (DC) were derived from X-ray examination, including thoracic-abdominal plain X-ray film, hilar computed tomography, high kV chest plain film or ultrafast CT, echocardiogram and angiography. Fontan operation was performed in 8 cases and anatomical correction in 7 with surgical exploration in 1 case. Thirteen patients survived, among whom 8 had complex cardiac defect and 5 of them received modified Fontan operation or total cavopulmonary connection (TCPC). CONCLUSIONS: Complex cardiac anomalies are not rare in mirror-image dextrocardia, and dextroversion and isolated dextrocardia were usually related to C-TGA+VSD+PS and TGA+CAVC respectively. X-ray examinations are of great significance in the diagnoses and classification of dextrocardia, and echocardiogram and ultrafast CT constitute the major modalities for clarifying intracardial defect and surgical indications. Fontan operations, including modified Fontan and total cavopulmonary connection, might be hopeful surgical treatment for complex congenital heart disease when indications are carefully evaluated.
Adolescent↗
Fetal dextrocardia: diagnosis and outcome in two tertiary centres.
OBJECTIVE: To evaluate the incidence of fetal dextrocardia, associated cardiac and extracardiac malformations, and outcome. DESIGN: Retrospective echocardiographic study. SETTING: Two tertiary centres for fetal cardiology. PATIENTS: 81 consecutive fetuses with a fetal dextrocardia presenting at Guy's Hospital, London, between 1983 and 2003 and at Hôpital Robert Debré, Paris, between 1988 and 2003. Fetal dextrocardia was defined as a condition in which the major axis of the heart points to the right. RESULTS: The incidence was 0.22%. There were 38 fetuses (47%) with situs solitus (SS), 24 (30%) with situs ambiguus (SA), and 19 (23%) with situs inversus (SI). Structural cardiac malformations were found in 25 cases (66%) of SS, 23 cases (96%) of SA, and 12 cases (63%) of SI. Extracardiac malformations were identified in 12 cases (31%) of SS, in five cases (21%) of SA, and in two cases (10%) of SI. Of the 81 cases of fetal dextrocardia, there were 27 interrupted pregnancies (15 of 24 SA, 10 of 38 SS, and 2 of 19 SI), six intrauterine deaths (3 of 38 SS, 2 of 24 SA, and 1 of 19 SI), and five neonatal deaths (3 of 24 SA, 1 of 19 SI, and 1 of 38 SS). There were 43 survivors (24 of 38 SS, 15 of 19 SI, and 4 of 24 SA). CONCLUSION: The majority of fetuses with dextrocardia referred for fetal echocardiography have associated congenital heart disease. There is a broad spectrum of cardiac malformation and the incidence varies according to the atrial situs. Fetal echocardiography enables detection of complex congenital heart disease so that parents can be appropriately counselled.
Chromosome Disorders↗
The course of the conduction system in dextrocardia.
This is a serial section examination of the conduction system of five hearts of various types of dextrocardia. In one case of dextroversion the conduction system was found to be normal. In one case of mirror-image dextrocardia the conduction system was normal but in the opposite spatial position. In one heart of mixed (discordant) dextrocardia, the sinoatrial node was in correct morphologic position. There were two atrioventricular nodes - a posterior node close to the coronary sinus and an anterior node in the roof of the morphologic right atrium. They formed two separate bundles which joined in the left ventricle to form a "sling". In two cases of presumptive mirror-image dextrocardia, with a common atrium in situs inversus according to the morphology of the atrial appendages, with common atrioventricular orifice (CAVO), the sinoatrial node was situated in wrong positions. Two atrioventricular nodes and bundles again formed a sling. There are various types of slings in discordant dextrocardia.
Atrioventricular Node↗
Diagnosis of isolated dextrocardia using angiocardiography or surgery.
BACKGROUND: Isolated dextrocardia is a rare phenomenon and usually associated with multiple cardiac anomalies. This study was to evaluate the accuracy of diagnosis of isolated dextrocardia by using angiocardiography and to compare it with the results of surgery. METHODS: The clinical data of 27 cases of congenital isolated dextrocardia were collected to understand the diagnostic approaches to the major cardiac anomalies. All cases underwent angiocardiography followed by palliative or curative surgery. The diagnosis was compared by angiocardiography relying on segmental analysis with the pathological features observed in surgery. RESULTS: The results of angiocardiography of 22 patients were the same as the pathological features observed during surgery, including one case with congenital left ventricular diverticulum was inadvertently omitted in angiocardiograhy. There were significantly dissimilar diagnoses between angiocardiograhy and post-operation in 5 patients, including anatomical corrected transposition of great arteries misinterpreted as corrected transposition of the great arteries in 1, complete transposition of great arteries misinterpreted as corrected transposition of the great arteries in 1, single ventricle misinterpreted as double-outlet right ventricle in 1, and anatomical double-outlet left ventricle misinterpreted as corrected transposition of the great arteries in 2. Misdiagnostic rate of angiocardiograhy was almost 20%. CONCLUSIONS: Angiocardiography is of great significance in the diagnosis and classification of isolated dextrocardia. However, because of the intricacy of cardiac anomalies of isolated dextrocardia, atrial angiography and double oblique projection are needed to improve the accuracy of diagnosis to support surgical treatments.
Adolescent↗
Mechanical reperfusion during acute myocardial infarction in a patient with dextrocardia.
Mechanical reperfusion is the preferred treatment for acute ST-elevation myocardial infarction, we describe a case of successful primary angioplasty and stenting in a patient with dextrocardia and situs inversus. Dextrocardia with complete situs inversus is a rare condition, occurring in about 2 in 10,000 live births. However, ischemic heart disease and myocardial infarction have been reported in patients with dextrocardia, and hence the coexistence of myocardial infarction and dextrocardia is not unusual. The incidence of atherosclerosis in this group is not known, but is considered to be the same as that in the general population. There have been a few reports of percutaneous coronary intervention in these patients. We describe a case of primary angioplasty and stenting in a patient with dextrocardia and situs inversus and the electrocardiographic correlation of successful myocardial reperfusion.
Angioplasty, Balloon, Coronary↗
Dextrocardia in pregnancy: 20 years' experience.
OBJECTIVE: To identify all pregnancies complicated by maternal dextrocardia and report the obstetric performance in these patients. STUDY DESIGN: A retrospective review of all deliveries between May 1984 and December 2004 at Prince of Wales Hospital, Chinese University of Hong Kong. Maternal demographic data as well as information on the antenatal course and delivery outcome were abstracted from the maternal records. Neonatal record review yielded information on the gestational age at delivery, birth weight, Apgar scores, sex and neonatal conditions after delivery. RESULTS: Fifteen singleton pregnancies in 9 patients with dextrocardia were identified. There were 6 pregnancies in 3 patients with situs inversus and 9 pregnancies in 6 patients with isolated dextrocardia. There were no apparent antenatal complications. None of the patients developed any cardiac symptoms antenatally. All the infants had a 5-minute Apgar score > 7. Four small-for-gestational-age (SGA) infants, defined after correcting for parity, gestational age, maternal height and maternal entry weight, occurred in patients with situs inversus. This is significantly different from the 10% expected (p < 0.002). No SGA infants were born to patients with isolated dextrocardia. CONCLUSION: Pregnancies complicated by maternal isolated dextrocardia did not have any detrimental effect on the disease or vice versa. However, SGA infants should be watched in patients with situs inversus.
Adult↗
Poland sequence with dextrocardia: which comes first?
We report on two cases of Poland sequence (defect of the pectoralis major and hand on the same side) with dextrocardia, and review the literature on such patients. In all 16 reported cases, the Poland defect was on the left side, and associated with a rib defect, whereas most cases of Poland sequence involve the right side, and few have a rib defect. The dextrocardia appeared to be a dextroposition, and was not associated with other cardiac defects, whereas isolated dextrocardias (without situs inversus) frequently are. These observations suggest that the dextrocardia associated with Poland sequence is usually secondary to it.
Dextrocardia↗
Radiofrequency catheter ablation of sustained intraatrial reentrant tachycardia in a patient with mirror-image dextrocardia.
INTRODUCTION: There has been limited experience with radiofrequency catheter ablation in patients with dextrocardia. This report describes a 37-year-old man who had secundum type atrial septal defect, mirror-image dextrocardia, and drug-refractory atrial tachycardia, in whom catheter-mediated radiofrequency energy successfully eliminated the tachycardia without complication. METHODS AND RESULTS: On electrophysiologic study, the atrial tachycardia could be induced and terminated by atrial extrastimulation or rapid atrial pacing. Mixed resetting response pattern and manifest entrainment were also demonstrated. These findings suggested that the mechanism of atrial tachycardia might be related to reentry with an excitable gap. Because of the mirror-image dextrocardia, biplane fluoroscopy was adjusted to the right anterior oblique (RAO) 60 degrees and left anterior oblique (LAO) 30 degrees positions. Inducibility of the tachycardia was completely abolished after the sixth application of radiofrequency current (30 W). It was noted that the successful electrogram preceded the onset of P wave during atrial tachycardia by about 40 msec. CONCLUSION: This report presents another case in which radiofrequency catheter ablation was used in a patient with dextrocardia. Atrial tachycardia in patients with congenital heart disease may be difficult to control pharmacologically; however, the use of radiofrequency catheter ablation could be recommended as an alternative to medication and surgery.
Adult↗
Coronary arteriography in patients with dextrocardia.
Coronary arteriography is described in a patient with dextrocardia and situs inversus totalis. Six cases of coronary arteriography in dextrocardia were reviewed from the literature, and 3 other cases were reported from the authors' institutions. In 9 of the 10 patients, coronary arteriography was performed without difficulty using standard Judkins or Sones catheters. Angiographers encountering patients with dextrocardia may use standard coronary arteriographic techniques with a few subtle modifications.
Aged↗
Thallium-201 myocardial SPECT in a patient with mirror-image dextrocardia and left bundle branch block.
A 53-year-old male patient with a previous diagnosis of situs inversus with mirror-image dextrocardia underwent thallium-201 (Tl-201) stress-redistribution myocardial perfusion single photon emission computed tomography (SPECT). Electrocardiogram (ECG) obtained on right hemithorax revealed constant complete left bundle branch block. Tl-201 stress-redistribution SPECT images revealed abnormal perfusion with reversible ischemia in the anteroseptal, septal and inferoseptal walls. Coronary angiography performed 1 month after SPECT study was normal. This case illustrates that false positive reversible perfusion defects can be seen in patients with mirror-image dextrocardia associated with constant complete left bundle branch block. To our knowledge, this is the first reported case of mirror-image dextrocardia and constant complete left bundle branch block with false positive Tl-201 SPECT findings.
Bundle-Branch Block↗
Diagnosis and outcome of dextrocardia diagnosed in the fetus.
A retrospective review of 5,539 fetal echocardiograms over a 22-year period revealed 85 cases of dextrocardia. In primary dextrocardia (46 cases), the incidence of situs solitus, inversus, and ambiguous, was similar and associated with a high incidence of complex cardiac malformations in situs solitus and situs ambiguous. Secondary dextrocardia (39 cases) was due to intrathoracic displacement and, when caused by diaphragmatic hernia, was associated with cardiac malformations in 31% of cases. Even in complex cases, fetal echocardiography was highly accurate; therefore, specific counseling can be given to parents.
British Columbia↗
Acute myocardial infarction in a patient with dextrocardia and situs inversus.
We report a case of 54-year-old man who presented to hospital with severe prolonged retrosternal chest pain of anginal nature. Electrocardiogram taken by his general practitioner showed minimal ST elevation in chest leads V1 and V2; there was also marked right axis deviation of P wave (negative in lead I and aVL) and of QRS complexes, together with low voltage in precordial leads V4 through V6 suggestive of dextrocardia. Repeat electrocardiogram with chest and limb leads reversed showed widespread, significant ST elevation in lead I, aVL, and V1 through V5 in keeping with extensive acute anterior myocardial infarction (MI). High cardiac enzymes and troponin level provided further confirmation. The extent of MI in such patients may be underestimated unless dextrocardia is timely recognized and leads reversed. We recommend that for patients with dextrocardia and situs inversus presenting with MI, both chest and limb leads be reversed to reveal the true extent of the infracted area.
Dextrocardia↗
Beating heart surgery in a patient with dextrocardia and complete situs inversus.
We present a 65-year-old female patient with dextrocardia and situs inversus who underwent successful coronary artery bypass without cardiopulmonary bypass. Vessels revascularized included right internal mammary artery to the left anterior descending artery and a saphenous vein graft to the first obtuse marginal branch. The procedure was performed on a beating heart through a median sternotomy with the use of a compression epicardial stabilizer. The patient was discharged to her home after an uneventful recovery. Only 12 similar cases of myocardial revascularization in patients with dextrocardia have been reported so far, and this is one of the first procedures, in patients with dextrocardia, performed off pump.
Abnormalities, Multiple↗
Coronary arterial revascularization in an adult with congenitally corrected transposition of great arteries and dextrocardia.
OBJECTIVES: Congenitally corrected transposition of great arteries with dextrocardia is an extremely rare lesion in adulthood. This group of patients does not live long enough for atherosclerotic coronary artery disease processes, because of existing comorbid anomalies. METHODS: We report a 47-year-old man with isolated congenitally corrected transposition of great arteries, dextrocardia, and athersclerotic coronary artery disease. The patient underwent coronary artery revascularization with cardiopulmonary bypass. The free left internal mammary artery (LIMA) was grafted to the tiny left anterior descending artery (LAD), and the reversed saphenous vein Y graft was anastomosed to the posterior descending and posterolateral branches of the morphologic right coronary artery. RESULTS: The patient recovered uneventfully. He is alive and well 24 months after the surgery. CONCLUSIONS: To our knowledge, the present case is the first congenitally corrected transposition of great arteries with dextrocardia treated with grafted coronary artery bypass. Early and full revascularization is very important for the systemic right ventricle exposed to a systemic workload. The vessel pathologies and technical details of this unusual case are discussed in this paper.
Coronary Disease↗