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2.
Rev. costarric. cardiol ; 23(1)jun. 2021.
Article in Spanish | LILACS, SaludCR | ID: biblio-1389032

ABSTRACT

Resumen Paciente masculino de 18 años, quien es valorado en el Servicio de Cardiología del Hospital Víctor Manuel Sanabria Martínez referido del primer nivel de atención por cuadro crónico de dolor torácico atípico acompañado de pectus excavatum y desplazamiento izquierdo del latido de punta a la exploración física. La radiografía de tórax posteroanterior evidencia una cardiomegalia moderada con levocardia máxima. El ecocardiograma transtorácico con función sistólica biventricular conservada, dilatación moderada del ventrículo derecho, sin signos de hipertensión pulmonar e imagen cardiaca de "corazón en lágrima". Angiotomografía computarizada de tórax con contraste que documenta malformación de la caja torácica con hundimiento de la región esternal, en relación con pectus excavatum. Corazón aumentado de tamaño en relación con cardiomegalia grado I y desplazamiento hacia el hemitórax izquierdo. No se observa pericardio en ninguna región. Aurícula derecha levemente dilatada con un ventrículo derecho con diámetro aumentado en su porción media y deformidad de su tracto de salida debido a la forma de la caja torácica, de tal manera que se corrobora el diagnóstico de una agenesia pericárdica completa.


Abstract An 18-year-old male patient who is evaluated in the Cardiology Department of the Víctor Manuel Sanabria Martínez Hospital, referred to the first level of attention due to chronic symptoms of atypical chest pain accompanied by pectus excavatum and left displacement of the peak beat. Chest X-ray Posteroanterior with moderate cardiomegaly with maximum levocardia. Transthoracic echocardiogram with preserved biventricular systolic function, moderate dilatation of the right ventricle, without signs of pulmonary hypertension and cardiac image of "tear heart". Thoracic Computed Angiotomography with contrast documenting malformation of the rib cage with sinking of the external region, in relation to pectus excavatum. Heart enlarged in relation to grade I cardiomegaly and displacement towards the left hemithorax. No pericardium is observed in any region. Slightly dilated right atrium with a right ventricle with an increased diameter in its middle portion and deformity of its outflow tract due to the shape of the rib cage. Corroborating the diagnosis of complete pericardial agenesis.


Subject(s)
Humans , Male , Adolescent , Pericardium/abnormalities , Cardiomegaly/diagnostic imaging , Levocardia/diagnostic imaging , Pericardium/diagnostic imaging
3.
Br J Radiol ; 92(1102): 20190231, 2019 Oct.
Article in English | MEDLINE | ID: mdl-31271542

ABSTRACT

Viscero-atrial situs encompasses the laterality, relative position and configuration of the abdominal viscera, the atria of the heart and the tracheobronchial tree. Determining the situs and cardiac position is the first step in the commonly used sequential, segmental approach to the imaging evaluation of congenital heart defects (CHD). Abnormalities of visceroatrial situs and cardiac position are frequently associated with the presence of complex CHDs and accurate assessment of situs abnormalities can help predict the probability and type of the defect. Multidetector CT (MDCT) angiography, with its multiplanar reformatting and volume rendering techniques, offers accurate information about the morphology and three-dimensional relationships of the various cardiac and extra cardiac structures. In this pictorial essay, we present the MDCT imaging findings of the spectrum of abnormalities of visceroatrial situs and cardiac position, using a third generation dual source CT scanner.


Subject(s)
Computed Tomography Angiography/methods , Heart Defects, Congenital/diagnostic imaging , Multidetector Computed Tomography , Viscera/diagnostic imaging , Bronchi/abnormalities , Bronchi/diagnostic imaging , Heart/embryology , Heart Atria/abnormalities , Heart Atria/diagnostic imaging , Heart Defects, Congenital/embryology , Humans , Levocardia/diagnostic imaging , Liver/abnormalities , Liver/diagnostic imaging , Stomach/abnormalities , Stomach/diagnostic imaging , Trachea/abnormalities , Trachea/diagnostic imaging , Viscera/abnormalities
6.
BMJ Case Rep ; 20152015 Dec 01.
Article in English | MEDLINE | ID: mdl-26628311

ABSTRACT

A 69-year-old man with situs inversus, levocardia and inverted great arteries developed severe dynamic left ventricular outflow tract (LVOT) obstruction with systolic anterior motion of the anterior mitral leaflet. There was no asymmetric septal hypertrophy. A possible mechanism of the LOVT obstruction in the present case may have been related to an abnormally long and bent outflow tract resulting from overriding of the right ventricle over the LVOT due to a congenital malposition of the heart. Mitral valve replacement with septal myectomy was performed in order to eliminate systolic anterior motion. The postoperative course has been excellent.


Subject(s)
Levocardia/complications , Levocardia/diagnostic imaging , Ventricular Outflow Obstruction/complications , Ventricular Outflow Obstruction/diagnostic imaging , Aged , Heart Ventricles/diagnostic imaging , Heart Ventricles/surgery , Humans , Levocardia/surgery , Male , Tomography, X-Ray Computed , Ventricular Outflow Obstruction/surgery
7.
J Radiol Case Rep ; 9(2): 16-23, 2015 Feb.
Article in English | MEDLINE | ID: mdl-25926924

ABSTRACT

When approaching a case with a situs abnormality, using the proper terminology, making a specific diagnosis, and understanding the other often associated abnormalities that need to be excluded are of great importance. We present a case of situs ambiguous in the presence of intestinal nonrotation and an obstructing duodenal web. Our patient initially presented at two days old with bilious emesis and failure to pass meconium after birth. An abdominal radiograph demonstrated an unusual bowel gas pattern, a reversed "double bubble" sign. A subsequent thorough imaging survey was crucial to further characterize our patient's unique anatomy. Overall, our case demonstrates many of the unusual plain radiographic and sonographic findings associated with our patient's situs abnormality and allows for review of situs abnormalities and their significance.


Subject(s)
Abnormalities, Multiple/diagnostic imaging , Duodenum/abnormalities , Intestines/abnormalities , Levocardia/diagnostic imaging , Stomach/abnormalities , Abnormalities, Multiple/surgery , Diagnosis, Differential , Duodenum/diagnostic imaging , Female , Humans , Infant, Newborn , Intestines/diagnostic imaging , Radiography , Stomach/diagnostic imaging , Ultrasonography
8.
Turk Kardiyol Dern Ars ; 42(6): 564-7, 2014 Sep.
Article in English | MEDLINE | ID: mdl-25362949

ABSTRACT

Anatomically corrected malposition of the great arteries (ACMGA) is defined as parallel arising of aorta and main pulmonary artery (PA) roots although ventriculoarterial connection is normal. Abnormally related aorta arises from the left ventricle, while abnormally related PA arises from the right ventricle. It can be diagnosed with via echocardiography. In some cases, additional imaging modalities such as computerized tomographic angiography and magnetic resonance are required. In this article, we presented two cases of ACMGA, 5-month-old boy and 1-month-old girl. We wanted to point out the importance of differential diagnosis of other great artery anomalies from this rare pathology.


Subject(s)
Levocardia/diagnosis , Transposition of Great Vessels/diagnosis , Diagnosis, Differential , Echocardiography , Female , Humans , Infant , Infant, Newborn , Levocardia/diagnostic imaging , Levocardia/surgery , Male , Radiography , Tomography , Transposition of Great Vessels/diagnostic imaging , Transposition of Great Vessels/surgery
9.
BMC Pregnancy Childbirth ; 13: 79, 2013 Mar 26.
Article in English | MEDLINE | ID: mdl-23530545

ABSTRACT

BACKGROUND: Fetal unilateral lung agenesis, complicated with cardiac shifting, is a rare anomaly, the diagnosis of which remains a challenge for many sonographers in routine screening programs. The present study describes a systematic approach for the diagnosis of fetal unilateral lung agenesis and cardiac malpositions in routine prenatal screening. METHODS: A total of 18 cases of fetal unilateral lung agenesis complicated with cardiac malposition were reviewed. A systematic method was proposed to identify the fetal left side and right side according to the fetal head position and posture by acquiring a long axis and transverse view of the fetus. Fetal unilateral lung agenesis was diagnosed by evaluation of the ipsilateral pulmonary artery. The diagnosis was confirmed by postnatal echocardiography, digital radiology, and computed tomography after birth or by autopsy findings. RESULTS: The left-sided fetal heart with the cardiac apex rotating to the left and posterior were confirmed in all 7 left lung agenesis cases, while the rightward shifting of the fetal heart together with the cardiac axis deviating to the right were confirmed in all 11 cases of right lung agenesis. The disappearance of the ipsilateral pulmonary artery was confirmed in all 18 cases of unilateral lung agenesis. Cardiac anomalies were present in a total of 7 of the 18 cases of lung agenesis with 4 of 7 in cases of left lung agenesis and 3 of 11 in cases of right agenesis. CONCLUSIONS: The systematic approach introduced in the current report is helpful in the diagnosis of fetal unilateral lung agenesis complicated with cardiac malposition. The information provided by this study may be helpful to better understand unilateral lung agenesis anatomically and to facilitate its potential examination.


Subject(s)
Abnormalities, Multiple/diagnostic imaging , Levocardia/diagnostic imaging , Lung Diseases/diagnostic imaging , Lung/abnormalities , Ultrasonography, Prenatal/methods , Echocardiography, Doppler, Color , Female , Humans , Lung/diagnostic imaging , Pregnancy , Pulmonary Artery/diagnostic imaging , Retrospective Studies
10.
Pediatr Cardiol ; 34(4): 1034-7, 2013 Apr.
Article in English | MEDLINE | ID: mdl-22614902

ABSTRACT

Isolated levocardia (ILC) is a developmental abnormality involving an abnormal abdominal situs with a normal cardiac situs. This abnormality is especially rare when it is associated with a normal cardiac anatomy. The prenatal diagnoses of seven cases were reported in the English literature. This report presents two cases referred to the authors' echocardiography laboratory for maternal diabetes mellitus in case 1 and suspected dextrocardia in case 2. In both cases, ILC with a structurally normal heart was diagnosed prenatally. The child in the first case was found to have a normal inferior vena cava (IVC) prenatally. Postnatally, he was found to have intestinal malrotation with duodenal obstruction and multiple splenules. Interruption of the IVC was shown by abdominal ultrasound. The child in the second case was found to have an interrupted IVC with azygos continuation prenatally. Postnatally, intestinal malrotation with no evidence of intestinal obstruction or asplenia was detected. Neither of the cases had reported cardiac arrhythmias. Early diagnosis is crucial in these cases due to the high incidence of associated anomalies and potential life-threatening conditions. Management of patients with ILC is dictated by the associated anomalies. Long-term follow-up assessment is recommended for these patients to monitor the development of rhythm abnormalities.


Subject(s)
Echocardiography , Levocardia/diagnostic imaging , Ultrasonography, Prenatal , Abnormalities, Multiple/diagnostic imaging , Adult , Electrocardiography , Female , Humans , Infant, Newborn , Male , Pregnancy
11.
Anadolu Kardiyol Derg ; 11(2): 131-6, 2011 Mar.
Article in English | MEDLINE | ID: mdl-21303758

ABSTRACT

OBJECTIVE: The aim of this study was to evaluate distinguishing the right/left side of the fetus, cardiac axis and position according to the standardized Cordes technique in 20 cases with cardiac malposition. METHODS: We studied retrospectively 1536 cases whose fetal echocardiographic examinations were performed between 1999 and 2006 in prenatal cardiology unit. Among these, cardiac malpositions were determined in 20 cases. The cardiac axis and position were determined according to the Cordes technique. All cases were followed-up by serial fetal echocardiograms until birth or intrauterine death occurred. In cases of intrauterine death, an autopsy was performed. After birth, physical and echocardiographic examinations were done and prenatal and postnatal diagnoses were compared. RESULTS: Of 1536 fetal echocardiograms performed, 144 revealed congenital heart diseases (9.4%), among these cases 20 were diagnosed with cardiac malposition. Of cases with cardiac malposition, 16 had congenital heart disease, and four had extracardiac malformation. There were six cases of isolated dextrocardia, three cases of situs inversus totalis, six cases of situs ambiguous, and one case of situs inversus with isolated levocardia. Of four cases with extracardiac malformations, two cases had mesoposition, one had dextroposition, and one had extreme levoposition. In six cases the autopsy findings were the same as that their prenatal echocardiographic findings. When postnatal echocardiographic results of the remaining cases with cardiac malposition due to congenital heart disease were compared with prenatal diagnoses, the same echocardiographic findings were verified. CONCLUSION: The fetal right/left axis must be determined correctly for the accurate diagnosis of cardiac malpositions. Therefore, we recommend that Cordes technique provides a simple and reliable determination of the fetal right/left axis and fetal situs.


Subject(s)
Dextrocardia/diagnostic imaging , Echocardiography/methods , Heart Defects, Congenital/diagnostic imaging , Heterotaxy Syndrome/diagnostic imaging , Situs Inversus/diagnostic imaging , Ultrasonography, Prenatal/methods , Dextrocardia/embryology , Female , Heart Defects, Congenital/embryology , Heterotaxy Syndrome/embryology , Humans , Levocardia/diagnostic imaging , Levocardia/embryology , Pregnancy , Retrospective Studies , Situs Inversus/embryology
12.
Cardiol Young ; 20(1): 60-5, 2010 Feb.
Article in English | MEDLINE | ID: mdl-20199706

ABSTRACT

OBJECTIVES: Heterotaxy syndrome, which is often associated with atrioventricular valvar regurgitation, has been considered a risk factor for the surgical repair for Fontan candidates. The results of atrioventricular valve repair in this challenging patient population remain largely unknown. METHODS: From July, 1994 to January, 2007, 25 patients with the heterotaxy syndrome consisting of 22 right isomerism and three left isomerism presented to the Shizuoka Children's Hospital Japan with significant (3-4+) atrioventricular valvar regurgitation necessitating atrioventricular valve repair. The clinical and Doppler/echocardiographic data were retrospectively reviewed to determine the efficacy of the repair and patient outcome. RESULTS: Patients were divided into two groups on the basis of atrioventricular valvar regurgitation at the most recent follow-up: those with a successful late outcome, (0-2) and those with a poor outcome (3-4). There were 17 (67%) patients with a successful outcome and 8 (33%) with a poor outcome. The repair technique including leaflet apposition was predictive of a successful outcome (p = 0.003). Overall survival was 64% (16/25). Survival was 88.2% (15/17) for patients with a successful result versus 12.5% (1/8) for those with a poor outcome (p = 0.0007). Of the 15 survivors, 13 have reached final completion of the Fontan circulation, and two currently remain at the bi-directional Glenn shunt stage. CONCLUSION: Atrioventricular valve repair can be accomplished in this challenging patient population with excellent results. The combination of the leaflet apposition technique and the Kay suture can be performed with an excellent outcome in the majority of patients with heterotaxy syndrome, even with significant atrioventricular valvar regurgitation.


Subject(s)
Heart Atria/abnormalities , Heart Defects, Congenital/surgery , Heart Valve Prosthesis Implantation/methods , Mitral Valve Insufficiency/surgery , Situs Inversus/diagnosis , Child , Dextrocardia/diagnostic imaging , Echocardiography, Doppler , Female , Follow-Up Studies , Fontan Procedure/methods , Heart Atria/surgery , Heart Defects, Congenital/diagnostic imaging , Heart Defects, Congenital/mortality , Heart Valve Diseases/diagnostic imaging , Heart Valve Diseases/surgery , Heart Valve Prosthesis , Humans , Levocardia/diagnostic imaging , Male , Mitral Valve Insufficiency/diagnostic imaging , Mitral Valve Insufficiency/mortality , Retrospective Studies , Risk Assessment , Survival Rate , Syndrome , Treatment Outcome
13.
Congenit Anom (Kyoto) ; 49(2): 56-60, 2009 Jun.
Article in English | MEDLINE | ID: mdl-19489955

ABSTRACT

Isolated levocardia (IL) is a rare condition of situs anomaly in which there is a normal left-sided heart (levocardia) with dextro position of the abdominal viscera. IL has been reported in children and adults with complex cardiac defects, whereas there are only few published reports regarding the prenatal diagnosis of IL. We report two prenatal cases of IL diagnosed by ultrasonography and magnetic resonance imaging (MRI). In both cases, fetal cardiac function remained within the normal range throughout pregnancy, and no treatment for the heart was required after birth. For the dextro position of abdominal viscera, one case was followed without any surgical procedure, but the other case required prophylactic operation due to malrotation of the small intestine. Although the prognosis of IL largely depends on the severity of associated cardiac anomaly, future bowel obstruction caused by intestinal malrotation may also be life-threatening. In this respect, prenatal diagnosis of IL is important, even when there is no associated cardiac structural anomaly. If IL is suspected in routine fetal ultrasonography, MRI may be recommended to obtain more detailed information on the anatomy of abdominal viscerae, and careful observation for bowel problems is required, especially after oral nutrition is started.


Subject(s)
Levocardia/diagnostic imaging , Levocardia/therapy , Magnetic Resonance Imaging , Ultrasonography, Prenatal , Adult , Female , Gestational Age , Humans , Infant, Newborn , Pregnancy , Pregnancy Outcome
15.
J Ultrasound Med ; 26(3): 361-5, 2007 Mar.
Article in English | MEDLINE | ID: mdl-17324986

ABSTRACT

OBJECTIVE: Isolated levocardia is a rare type of situs inversus in which the heart is in the normal levo position, but the abdominal viscera are in the dextro position. We aim to describe our experience with prenatal diagnosis and management in fetuses with isolated levocardia. METHODS: Of all the cases referred to our tertiary ultrasound unit, 3 cases of isolated levocardia were diagnosed. Patients and fetuses were evaluated every 4 weeks until delivery and postnatally. RESULTS: Two of the 3 fetuses had interruption of the inferior vena cava with azygous continuation. However, postnatal evaluation revealed polysplenia in 1 neonate and asplenia in another. Polysplenia was also diagnosed in the third neonate, who had a normal inferior vena cava on antenatal examination. One neonate had a small ventricular septal defect. CONCLUSIONS: Fetal isolated levocardia is associated with a good outcome, in which other malformations are excluded. Therefore, we suggest conservative management in such cases.


Subject(s)
Levocardia/diagnostic imaging , Ultrasonography, Prenatal , Vena Cava, Inferior/abnormalities , Viscera/diagnostic imaging , Adult , Female , Humans , Pregnancy , Pregnancy Outcome , Viscera/abnormalities
16.
J Am Soc Echocardiogr ; 16(7): 732-7, 2003 Jul.
Article in English | MEDLINE | ID: mdl-12835659

ABSTRACT

BACKGROUND: To date there is no direct comparison of transesophageal and transthoracic Doppler tissue data in patients with cardiac malposition. We compared data acquired from both methods in an animal model. METHOD: We studied 10 15-kg Yorkshire pigs during incremental atrial pacing to modify myocardial responses. Peak isovolumic velocity (IVV) and isovolumic acceleration (IVA) during isovolumic contraction, ejection (S wave), and diastolic velocities (early passive and active filling, E and A wave, respectively) were measured. RESULTS: Bland-Altman plots showed comparable values for IVA, IVV, and E velocity using both methods. Measurements of A velocities were significantly different. S wave velocity measurements were significantly different in animals with cardiac malposition but not in those with levocardia. Malposition did not significantly effect measurements of IVA or IVV by either technique. CONCLUSION: Assessment of systolic function using the indices IVA and IVV by tranesophageal and transthoracic echocardiography are comparable. The tranesophageal assessment of velocities associated with greater degrees of myocardial shortening or lengthening cannot be directly compared with measurements derived from a transthoracic approach in patients with cardiac malposition.


Subject(s)
Echocardiography, Doppler , Echocardiography, Transesophageal , Animals , Cardiac Pacing, Artificial , Echocardiography , Echocardiography, Doppler/methods , Levocardia/diagnostic imaging , Myocardial Contraction/physiology , Swine
18.
JBR-BTR ; 84(1): 1-4, 2001 Feb.
Article in English | MEDLINE | ID: mdl-11307871

ABSTRACT

A rare case of an asymptomatic heterotaxy syndrome associated with dextrocardia is presented in a 45-year-old woman. This anomaly was incidentally discovered on computed tomography and ultrasound of the upper abdomen. Besides the presence of multiple abnormally positioned spleens, right-sided stomach, left-sided liver, short pancreas and venous anomalies, like interrupted left-sided inferior vena cava with azygos continuation, and dextrocardia were seen. Although cardiac abnormalities are frequently associated with heterotaxy syndromes with polysplenia in children, this is far less frequent in adults because of early decease in children with cardiac abnormalities. To the best of our knowledge, this is the third reported case of heterotaxy with polysplenia associated with dextrocardia in an asymptomatic adult patient.


Subject(s)
Situs Inversus/diagnostic imaging , Spleen/abnormalities , Female , Humans , Levocardia/diagnostic imaging , Middle Aged , Spleen/diagnostic imaging , Syndrome , Tomography, X-Ray Computed , Viscera/abnormalities , Viscera/diagnostic imaging
19.
Cardiol Young ; 10(4): 396-404, 2000 Oct.
Article in English | MEDLINE | ID: mdl-10950338

ABSTRACT

BACKGROUND: The morphological definition of atrial chambers, and the determination of atrial laterality, are based on analysis of the structure of the atrial appendages. The systemic and pulmonary venous connections to the heart, nonetheless, are important in the management of patients having isomeric appendages. In this study, therefore, we analysed the morphology of the postero-superior walls of the atrial chambers so as to provide evidence concerning the morphogenetic background of those hearts, and to improve operative management. METHODS: We reviewed 15 autopsied specimens with isomeric right appendages, and 10 with isomeric left appendages, paying particular attention to the morphology of the systemic and pulmonary venous connections. The postero-superior walls of the atrial chambers can be made up of the atrial body, the systemic venous components, or the pulmonary venous component. We analysed the contributions made by each of these components. RESULTS: The postero-superior walls of the atrial chambers were markedly variable, but could be grouped into five patterns. Bilaterally well-developed systemic venous components and absence of the pulmonary venous component within the hypoplastic atrial body were present in 9 hearts with extracardiac pulmonary venous connections in the setting of right isomerism. Bilaterally well-developed systemic venous components, and a hypoplastic pulmonary venous component within the hypoplastic atrial body, were present in 5 hearts with intracardiac pulmonary venous connections in right isomerism. Bilaterally well-developed systemic venous components, and a hypoplastic pulmonary venous component within the sizable atrial body, were present in 1 heart with an intracardiac pulmonary venous connection in right isomerism. A well-developed pulmonary venous component within the atrial body, and hypoplasia of one systemic venous component, were present in 7 hearts with left isomerism. A well-developed pulmonary venous component within the atrial body, and hypoplasia of bilateral systemic venous components, were present in 3 hearts with left isomerism. CONCLUSIONS: The postero-superior walls of the atrial chambers in hearts with isomeric atrial appendages can be analysed on the basis of a compound structure made of bilateral systemic venous components, a central pulmonary venous component, and the body of the atrium. Hearts with isomeric right appendages have absence or hypoplasia of the pulmonary venous component, while hearts with isomeric left appendages have hypoplastic systemic venous components.


Subject(s)
Heart Atria/pathology , Levocardia/pathology , Pulmonary Veins/pathology , Autopsy , Bronchi/embryology , Bronchi/pathology , Echocardiography , Female , Fetus/embryology , Fetus/pathology , Heart Atria/diagnostic imaging , Heart Atria/embryology , Humans , Infant, Newborn , Levocardia/diagnostic imaging , Levocardia/embryology , Male , Pulmonary Veins/diagnostic imaging , Pulmonary Veins/embryology , Situs Inversus/embryology , Situs Inversus/pathology , Spleen/embryology , Spleen/pathology
20.
Surg Radiol Anat ; 21(2): 143-5, 1999.
Article in English | MEDLINE | ID: mdl-10399216

ABSTRACT

Isolated levocardia is a rare condition in which intestinal malrotation may be encountered. The case herein reported is particularly uncommon and raises a number of questions about the development of abdominal symmetry.


Subject(s)
Levocardia/pathology , Situs Inversus/pathology , Child, Preschool , Female , Humans , Levocardia/diagnostic imaging , Levocardia/embryology , Radiography , Situs Inversus/diagnostic imaging , Situs Inversus/embryology
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