Severe COVID-19 acute respiratory distress syndrome in an adult with single-ventricle physiology: a case report.

BACKGROUND: COVID-19 can induce acute respiratory distress syndrome (ARDS). In patients with congenital heart disease, established treatment strategies are often limited due to their unique cardiovascular anatomy and passive pulmonary perfusion. CASE PRESENTATION: We report the first case of an adult with single-ventricle physiology and bidirectional cavopulmonary shunt who suffered from severe COVID-19 ARDS. Treatment strategies were successfully adopted, and pulmonary vascular resistance was reduced, both medically and through prone positioning, leading to a favorable outcome. CONCLUSION: ARDS treatment strategies including ventilatory settings, prone positioning therapy and cannulation techniques for extracorporeal oxygenation must be adopted carefully considering the passive venous return in patients with single-ventricle physiology.

A case report of late-onset symptoms of erythrocytosis in univentricular dextrocardia.

RATIONALE: Univentricular dextrocardia is a rare congenital heart disease that usually presents cyanotic manifestations from childhood. Due to the sustained dysfunction of blood oxygenation, it is very difficult to keep an asymptomatic survival. Herein, we described an interesting case of univentricular dextrocardia who suffered from initial symptoms in his middle age. PATIENT CONCERNS: A 54-year-old male patient with numbness and tingling of limbs was admitted to hospital due to the secondary manifestations of congenital heart disease. DIAGNOSIS: The patient was diagnosed as univentricular dextrocardia with pulmonary hypertension and secondary erythrocytosis based on computed tomography (CT) scan, echocardiography, and laboratory examinations. INTERVENTIONS: Intravenous hydration therapy with normal saline successfully eliminated his hyperviscosity associated symptoms. In view of socio-economic reasons, this patient refused surgical evaluation and further medical interventions. OUTCOMES: During 18-month follow up, he received no drug except for regular water intake. Fortunately, his life quality was satisfactory, and no other symptoms emerged except for mild numbness of limbs. LESSONS: In univentricular dextrocardia, it is possible to keep a long-term asymptomatic period due to the slow progress of pathophysiology. In this population, regular cardiac function evaluation and avoiding dehydration may help improve the quality of life.

Dextrocardia with complete AV block and the implantation of a temporary pacemaker before cesarean section: A case report.

RATIONALE: Patients with situs inversus totalis (SIT) and complete atrioventricular (AV) block are extremely rare, and only few cases have been reported up to now. Due to the rarity of such condition and its anatomical complexity, we reported this case as a reference for obstetricians, providing valuable insights into potential clinical treatment. PATIENT CONCERNS: We reported a case of 30-year-old patient with complete AV block, and her heart rate was only about 45 beats per minute. DIAGNOSES: The patient was diagnosed with term delivery with SIT and complete AV block. INTERVENTIONS: A temporary pacemaker (TPM) was implanted before cesarean section (CS) because of complete AV block. OUTCOMES: CS was performed successfully after the implantation of a TPM. The heart rate remained stable, and the 24-hour blood loss was limited. LESSONS: This study presents a complex heart disease case which needed more frequent antenatal examination and restriction of physical activity. To reduce the risk of pregnancy, basic disease needed to be cured before pregnancy. Our findings could provide guidance for future clinical studies.

Bloqueio atrioventricular total em dextrocardia e doença de Chagas: implante de marcapasso dupla-câmara com upgrade para estimulação biventricular / Complete atrioventricular block in dextrocardia and Chagas disease: implant of a dual chamber pacemaker with upgrade to biventricular stimulation

A dextrocardia é uma anomalia rara e sua associação ao bloqueio atrioventricular total é ainda maisincomum. A cardiopatia chagásica crônica é uma doença endêmica no Brasil, onde o aparecimento dessaarritmia é relativamente frequente (forma arritmogênica). Relatamos o caso de uma paciente de 38 anos de idade submetida a implante de marcapasso dupla-câmara por bloqueio atrioventricular total, portadora de cardiopatia chagásica crônica, que, em seu acompanhamento, apresentou insuficiência cardíaca refratária e necessidade de ressincronização cardíaca, evoluindo com melhora da classe funcional em sua avaliação precoce.

Dextrocardia is a rare anomaly, and its association with complete atrioventricular block is even moreunusual. Chronic Chagasic heart disease is endemic in Brazil, where the onset of this arrhythmia is relatively frequent (arrhythmogenic form). We report a 38-year-old patient with Chagasic heart disease undergoing implantation of a dual chamber pacemaker for complete atrioventricular block, who developed refractory heart failure during the follow-up and required cardiac resynchronization, evolving with improved functional class in the early assessment.

An adult case of polysplenia syndrome associated with sinus node dysfunction, dextrocardia, and systemic venous anomalies.

A 54-year-old woman was referred to our hospital for symptomatic sinus bradyarrhythmia with a sinus pause of 8 seconds. She was diagnosed with dextrocardia during childhood and discovered to have heterotaxy syndrome when she had an appendectomy during her teenager years. Chest and abdominal examinations by computed tomography showed multiple spleens located on the right side and abnormal drainages of the superior and inferior vena cava. Left isomerism was diagnosed by bilaterally bilobed lungs. Because of a patent bilateral superior vena cava, pacemaker leads were implanted using the right cephalic vein approach. Her fainting symptoms disappeared after pacemaker implantation.

Incidental finding of complete situs inversus in a polytraumatized adult.

BACKGROUND: Situs inversus is a rare abnormality typically posing a diagnostic dilemma during routine evaluation of acute abdominal emergencies such as in acute appendicitis and cholecystitis. It is rare to detect such in the setting of trauma. OBJECTIVE: To report an incidental finding of complete situs inversus in a poly-traumatized adult. METHODS: The clinical records of the patient including preoperative evaluation, intra-operative findings and postoperative care were reviewed. RESULT: A 53 year old man presented with difficulty breathing, left sided chest pain, generalized abdominal pain and distension 18 hours after a vehicular road traffic accident. Examination revealed features of left sided haemothorax, absent heart sounds, generalized peritonitis and limb injuries. Plain chest radiograph confirmed left haemothorax with dextrocardia. He had a left closed thoracostomy tube drainage and exploratory laparotomy which revealed complete situs inversus of intra-abdominal organs alongside a jejunal perforation which was repaired. Postoperative recovery was uneventful. CONCLUSION: Complete situs inversus is uncommon and may not be anticipated in evaluation of trauma patients. Preoperative clinical and radiological evaluation may however be helpful in making a pre-operative diagnosis and further management.

Transvenous permanent pacemaker implantation in dextrocardia: technique, challenges, outcome, and a brief review of literature.

AIMS: Dextrocardia is a rare congenital anomaly. Pacemaker implantation in dextrocardia can be challenging because of the distorted anatomy and associated anomalies. The literature regarding implantation of pacemaker in dextrocardia is scarce. METHODS AND RESULTS: The study involved retrospective analysis of records of patients with dextrocardia who had undergone pacemaker implantation between January 2006 and July 2013 from a single centre. Six patients with dextrocardia (five males and one female) underwent permanent pacemaker implantation (PPI) between January 2006 and July 2013. Of them, three had situs solitus dextrocardia and three situs inversus dextrocardia. All three patients with situs solitus dextrocardia had associated corrected transposition of great arteries. The indication for pacemaker implantation was symptomatic complete atrioventricular (AV) block in four, high-grade AV block in one, and sinus node dysfunction in one patient. A favourable outcome was noted during a mean follow-up of 3.9 years (4 months to 7 years) with one patient needing a pulse generator replacement. CONCLUSION: Permanent pacemaker implantation in dextrocardia can be challenging because of the distorted anatomy. Use of a technique employing angiography to delineate chamber anatomy and relationship can assist the operator during such difficult PPIs. The medium- and long-term survival after a successful pacemaker implantation in dextrocardia is favourable.

Catheter ablation of persistent atrial fibrillation in a patient with dextrocardia.

Dextrocardia is a rare anomaly where the heart is located on the right side of the chest instead of the normal left side. Ablation of atrial fibrillation (AF) with such an inverted anatomy may be challenging for the manipulation of the catheters. Here we report a case of dextrocardia who underwent ablation for persistent AF guided by image integration system.

Un insólito caso de síndrome de Kartagener / An unusual case of kartagener syndrome

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Initial experience with the Amplatzer Vascular Plug IV in congenital heart disease: coronary artery fistula and aortopulmonary collateral artery embolization.

BACKGROUND: A number of percutaneous devices are available to embolize anomalous vessels in congenital heart disease. We report our initial single-center experience with the new Amplatzer Vascular Plug IV (AVP IV) in congenital heart disease to embolize a coronary artery fistula and aortopulmonary collateral arteries in 4 cases. METHODS: From August 2009 until April 2010, 7 AVP IV devices were deployed in 4 patients, age range 5 months to 9 years, weight 3.5-27.7 kg. One patient had a large coronary artery fistula, the others had anomalous aortopulmonary collaterals; 2 patients had tetralogy of Fallot with pulmonary atresia, with the other having dextrocardia, anomalous pulmonary venous drainage and pulmonary atresia. RESULTS: In all 4 patients, vessels intended to be closed with the AVP IV were closed successfully with minimal residual shunting and no device failures. Deployed devices ranged from 4-8 mm in diameter. One patient had 4 devices, closing large branching infradiaphragmatic aortopulmonary collaterals. The other 3 patients had single devices. Complete vessel embolization was seen with no device embolization or implantation complication. CONCLUSION: This preliminary experience illustrates the utility of the AVP IV in congenital heart disease, occluding a coronary artery fistula and aorto-pulmonary collaterals, with devices between 4 mm and 8 mm in diameter, demonstrating its safety and effectiveness. It is particularly useful in embolizing difficult-to-reach large, tortuous vessels with a small-sized catheter in a single procedure. Longer-term follow up in a larger cohort of patients will be required to establish long-term efficacy and device safety.

Resynchronization therapy transvenous approach in dextrocardia and congenitally corrected transposition of great arteries.

Cardiac resynchronization therapy (CRT) is an acknowledged treatment for advanced heart failure in acquired dilated cardiomyopathy, resistant to pharmacotherapy. Although there are no therapeutic standards regarding heart failure originating from congenital heart defects with systemic right ventricle, a number of CRT implantations by transvenous approach in congenitally corrected transposition of the great arteries (CCTGA) have been reported since 2001, even though none of them expressly referred to a case concomitant with dextrocardia and situs inversus anomaly. We present a 57 year-old patient with dextrocardia and CCTGA, who underwent surgical closure of interatrial and interventricular septal defects at the age of 19, and in whom a VVI pacemaker was subsequently implanted at age 36. A three-lead CRT system was implanted by transvenous approach. Imaging techniques, including multi-slice computed tomography, targeted to pacing system and unusual anatomical relationships were applied. Within a 20-month follow-up, a significant improvement of functional NYHA class, systemic right ventricle ejection fraction and exercise capability were observed. Entirely transvenous CRT system implantation is feasible in patients with dextrocardia and CCTGA, and has substantial potential for long-term benefits.

Primary angioplasty and later elective multivessel stenting in a patient with dextrocardia: a case report and literature review.

Dextrocardia is a rare congenital anomaly with a prevalence of 1 in 10,000 births. The incidence of coronary artery disease in such patients is thought to be similar to that of the general public, however, patients are seldom seen during routine clinical practice and at the cardiac catheterization laboratory. Patients with this condition and acute myocardial ischemia may pose challenges at presentation, clinical and diagnostic findings and at cardiac catheterization. In this report, a male patient presenting with acute inferior myocardial infarction and three-vessel coronary artery disease was managed successfully with emergency primary angioplasty and later elective multivessel stenting. This is the first combined primary and later multivessel stenting to be reported in a single patient with dextrocardia and situs inversus. The clinical findings, details of the procedures and literature review will be discussed.

Transvenous cardiac resynchronization therapy in complex congenital heart diseases: dextrocardia with transposition of the great arteries after Mustard operation.

Cardiac resynchronization therapy revealed first promising results in patients with a congenital heart disease and a systemic right ventricle. Contrast-enhanced magnetic resonance imaging showed accessibility of the coronary sinus in an 18-year-old male patient with mirror dextrocardia, d-transposition of the great arteries and ventricular septal defect (VSD) after Mustard operation and VSD patch closure. In literatures, transvenous lead placement is discussed in this anatomical setting, with opposed position of the ventricular leads and reliable lead characteristics.

Percutaneous mitral commissurotomy in a case of mirror-image dextrocardia and rheumatic mitral stenosis.

A 43-year-old male with mirror-image dextrocardia and severe rheumatic mitral stenosis was subjected to successful percutaneous transvenous mitral commissurotomy (PTMC). The standard Inoue technique was modified by transseptal catheterization via the left femoral vein, image inversion, delineation of the interatrial septal anatomy via levophase pulmonary angiography, septal contrast staining and pigtail catheter insertion in the noncoronary aortic sinus, interatrial septal puncture with the transseptal needle rotated to a 7 o'clock position and left ventricular entry with a reverse loop technique. There were no procedural complications. Intracardiac pressures and mitral valvular planimetry suggested a successful procedural outcome. This case illustrates that PTMC can be accomplished safely in patients with this unusual cardiac anatomy with a few modifications in the standard technique.