-
Cureus Apr 2024Kartagener's syndrome is a genetic condition that is caused by a triad of symptoms, which includes bronchiectasis, chronic sinusitis, and situs inversus, and is...
Kartagener's syndrome is a genetic condition that is caused by a triad of symptoms, which includes bronchiectasis, chronic sinusitis, and situs inversus, and is considered rare. It is caused by defective ciliary motility, leading to impaired mucociliary clearance. We report a case of a 24-year-old male who presented with primary infertility and a long-standing history of recurrent respiratory infections. Physical examination revealed dextrocardia and digital clubbing. Radiological investigations confirmed situs inversus totalis and bronchiectasis. Semen analysis revealed azoospermia. Genetic analysis was not done due to financial constraints. However, the constellation of clinical features was diagnostic of Kartagener's syndrome. The patient was managed with antibiotics and chest physiotherapy. In vitro fertilization (IVF) was advised for infertility. A successful pregnancy was achieved through IVF, indicating viable sperm despite immotility. The aforementioned case underscores the significance of maintaining a heightened sense of suspicion for Kartagener's syndrome in individuals exhibiting unexplained bronchiectasis and infertility. Early diagnosis can prevent chronic respiratory morbidity and enable parenthood through assisted reproduction.
PubMed: 38770502
DOI: 10.7759/cureus.58635 -
Clinical Medicine Insights. Case Reports 2024Kartagener's syndrome, a rare autosomal recessive genetic disorder, is characterized by primary ciliary dyskinesia (PCD), resulting in defective cilia function in the...
BACKGROUND
Kartagener's syndrome, a rare autosomal recessive genetic disorder, is characterized by primary ciliary dyskinesia (PCD), resulting in defective cilia function in the respiratory tract and fallopian tubes.
CASE PRESENTATION
This case report discusses a 23-year-old female with Kartagener's syndrome, bronchiectasis, and cardiac involvement, who presented with shortness of breath, cough, and syncope. Notably, she received home oxygen therapy but became exhausted, leading to loss of consciousness. Clinical examination revealed prominent heart sounds and abnormal lung findings. Laboratory results indicated leukocytosis, and an ECG confirmed dextrocardia and cardiac abnormalities. Doppler studies identified mitral and tricuspid regurgitation along with severe pulmonary arterial hypertension. Antibiotics were administered for coagulase-negative Staphylococcus infection. The patient improved with a treatment regimen, including oxygenation and nebulization. Regular follow-up and patient education were emphasized.
CONCLUSION
This case underscores the complexity of Kartagener's syndrome and the importance of a multidisciplinary approach in managing its respiratory and cardiac manifestations.
PubMed: 38706639
DOI: 10.1177/11795476241251940 -
Scientific Reports May 2024The main objective of this study was to investigate the incidence and characteristics of electrocardiographic abnormalities in patients with microtia, and to explore...
The main objective of this study was to investigate the incidence and characteristics of electrocardiographic abnormalities in patients with microtia, and to explore cardiac maldevelopment associated with microtia. This retrospective study analyzed a large cohort of microtia patients admitted to Plastic Surgery Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, from September 2017 to August 2022. The routine electrocardiographic reports of these patients were reviewed to assess the incidence and characteristics of abnormalities. The study included a total of 10,151 patients (5598 in the microtia group and 4553 in the control group) who were admitted to the Plastic Surgery Hospital of Peking Union Medical College. The microtia group had a significantly higher incidence of abnormal electrocardiographies compared to the control group (18.3% vs. 13.6%, P < 0.01), even when excluding sinus irregularity (6.1% vs. 4.4%, P < 0.01). Among the 1025 cases of abnormal electrocardiographies in the microtia group, 686 cases were reported with simple sinus irregularity. After excluding sinus irregularity as abnormal, the most prevalent abnormalities was right bundle branch block (37.5%), followed by sinus bradycardia (17.4%), ST-T wave abnormalities (13.3%), atrial rhythm (9.1%), sinus tachycardia (8.3%), and ventricular high voltage (4.7%). Less common ECG abnormalities included atrial tachycardia (2.1%), ventricular premature contraction (2.4%), and ectopic atrial rhythm (1.8%). atrioventricular block and junctional rhythm were present in 1.2% and 0.9% of the cases, respectively. Wolff Parkinson White syndrome and dextrocardia had a lower prevalence, at 0.6% and 0.9%, respectively. The occurrence of electrocardiographic abnormalities in microtia patients was found to be higher compared to the control group. These findings highlight the potential congenital defect in cardiac electrophysiology beyond the presence of congenital heart defect that coincide with microtia.
Topics: Humans; Congenital Microtia; Male; Female; Electrocardiography; Retrospective Studies; Adolescent; Child; Adult; Young Adult; Incidence; Arrhythmias, Cardiac; China
PubMed: 38702362
DOI: 10.1038/s41598-024-60610-9 -
Pediatrics and Neonatology Apr 2024Previous studies on congenital heart diseases (CHD) associated with dextrocardia were based on selective patient databases and did not reflect the full spectrum of...
BACKGROUND
Previous studies on congenital heart diseases (CHD) associated with dextrocardia were based on selective patient databases and did not reflect the full spectrum of dextrocardia in the general population. Additionally, these studies had complex classification and presentation. Nor did these studies elaborate on the distribution of the associated CHD's complexity, the various segmental connections, and associated CHD among the four visceroatrial situs.
METHODS
We retrospectively reviewed the medical records of 211 children with primary dextrocardia. We used a segmental approach to diagnose CHD. We then analyzed and compared the distribution of the above-mentioned issues among the four visceroatrial situs.
RESULTS
Dextrocardia occurred most commonly with situs inversus (52.6%), followed by situs solitus (28.4%), asplenia (17.1%), and polysplenia (1.9%). Although some patients had a structurally normal heart (22.7%) or they were associated with simple CHD (17.5%), most patients had complex CHD (59.7%) consisting of a single ventricle (34.6%) or conotruncal anomaly (25.1%) (double-outlet right ventricle [7.6%], corrected transposition of the great arteries [6.2%], complete transposition of the great arteries [5.7%], tetralogy of Fallot [4.7%], etc.). Situs inversus or polysplenia had a higher prevalence of a structurally normal heart or associated with simple CHD, two patent atrioventricular (AV) valves connections, and biventricular AV connections. Situs solitus or asplenia had a higher prevalence of associated complex CHD, common AV valve connection, univentricular AV connection, pulmonary outflow tract obstruction, and anomalous pulmonary venous drainage.
CONCLUSION
Our study finds that situs inversus is the most common visceroatrial situs in dextrocardia. Although some patients had a structurally normal heart or were associated with simple CHD, most patients have associated complex CHD consisting of a single ventricle or conotruncal anomaly. Dextrocardia is associated with a higher incidence of complex CHD in situs solitus and asplenia groups than in situs inversus and polysplenia groups.
PubMed: 38692948
DOI: 10.1016/j.pedneo.2023.10.011 -
Clinical Case Reports May 2024Situs inversus totalis is a rare diagnosis, but the likelihood of experiencing myocardial infarction is presumed to be comparable to the general population average. In...
Situs inversus totalis is a rare diagnosis, but the likelihood of experiencing myocardial infarction is presumed to be comparable to the general population average. In individuals exhibiting situs inversus with suspected myocardial infarction, ECG recording, including right precordial leads, is crucial for diagnostic assessment. Coronary angiography and intervention should be performed with standard equipment using inverted maneuvers and radiographic projections.
PubMed: 38681040
DOI: 10.1002/ccr3.8768 -
The Journal of Tehran Heart Center Oct 2023Pregnancy after a heart transplant is a concern for many female recipients, and it remains a medical challenge that raises many questions. A 24-year-old woman, gravida...
Pregnancy after a heart transplant is a concern for many female recipients, and it remains a medical challenge that raises many questions. A 24-year-old woman, gravida 3, para 0, contacted us for obstetric care in the first trimester of gestation, about 3 years after an orthotopic cardiac transplant. She was a known case of dextrocardia with congenitally corrected transposition of the great arteries. The transplant had been performed for severe retractable heart failure, manifesting during her previous lost pregnancy. The course of the current gestation was uneventful. The patient's cardiovascular function was good throughout the pregnancy. She was admitted to the hospital for dyspnea at 35 weeks and 4 days of gestational age. A cardiovascular consult and echocardiography were performed. The results were within the normal range, and labor pains explained the patient's dyspnea. Because of labor pain, a repeat Cesarean section was performed at 35 weeks and 4 days of gestational age. A male baby weighing 2700 g, with an Apgar score of 7/9, was delivered. The patient was discharged after delivery with enoxaparin (40 mg), prednisolone (5 mg), levothyroxine, and mycophenolate mofetil (500 mg) without any complications. Follow-up was carried out at 1 month, and no abnormality was found. Here, we report a case of a successful pregnancy in a young woman after a heart transplant.
PubMed: 38680644
DOI: 10.18502/jthc.v18i4.14829 -
Clinical Case Reports Apr 2024A 46-year-old woman with congenitally corrected transposition of the great arteries (ccTGA) associated with dextrocardia, situs viscerus inversus, and left superior vena...
Atrial tachycardia ablation through the sub-pulmonary ventricle in a patient with multiple malformations associated with congenitally corrected transposition of the great arteries and double-sided slow-pathway.
A 46-year-old woman with congenitally corrected transposition of the great arteries (ccTGA) associated with dextrocardia, situs viscerus inversus, and left superior vena cava persistence presented with an incessant supraventricular tachycardia. Electrophysiological study was not conclusive in differential diagnosis of atrial tachycardia versus atypical atrioventricular (AV) nodal reentrant tachycardia, also due to the unconventional anatomy of the coronary sinus. By a comprehensive mapping of cardiac chambers, a double side slow-pathway was localized in both atrial chambers and subsequently ablated by radiofrequency delivery without tachycardia changes. Aortic root and cusps were devoid of electrical activity. The muscular part of the sub-pulmonary ventricle at the level of interatrial septum showed an earliest activation signal of -90 ms and ablation of this site was effective in abolish the tachycardia. This is the first case to report technical concerns of septal atrial tachycardia ablation in ccTGA associated with multiple anatomical malformations. Moreover, some peculiarities have been reported for the first time including the presence of double-side AV nodal slow-pathway and atypical localization of the tachycardia origin into the muscular part of the sub-pulmonary ventricle instead of posterior pulmonary cusp.
PubMed: 38659502
DOI: 10.1002/ccr3.8745 -
BMC Cardiovascular Disorders Apr 2024During normal sinus rhythm, atrial depolarization is conducted from right atrium to left atrium through Bachmann's bundle, and a normal P wave axis which is measured on...
BACKGROUND
During normal sinus rhythm, atrial depolarization is conducted from right atrium to left atrium through Bachmann's bundle, and a normal P wave axis which is measured on the frontal plane is between 0º and + 75º. The change of P wave polarity is helpful for the analysis of origin point.
CASE PRESENTATION
We report a patient with negative P wave in lead I. The characteristics of QRS complex in leads V to V are helpful to preliminarily differential diagnosis. The 12-lead electrocardiogram (ECG) with correct limb leads (right arm-left arm) placement shows sinus rhythm with complete right bundle branch block (RBBB).
CONCLUSIONS
The change of P wave polarity as well as characteristics of QRS complex can help identify limb-lead reversals.
Topics: Humans; Electrocardiography; Bundle-Branch Block; Sinoatrial Node; Heart Atria; Atrioventricular Node
PubMed: 38643100
DOI: 10.1186/s12872-024-03875-2 -
World Journal of Critical Care Medicine Mar 2024Mucormycosis is a rare, rapidly progressive and often fatal fungal infection. The rarity of the condition lends itself to unfamiliarity, delayed treatment, and poor...
BACKGROUND
Mucormycosis is a rare, rapidly progressive and often fatal fungal infection. The rarity of the condition lends itself to unfamiliarity, delayed treatment, and poor outcomes. Diagnosis of fungal infections early enough to enable appropriate treatment occurs in less than half of affected patients.
CASE SUMMARY
An 11-year-old girl with a history of 15% total body surface area scald burns involving both lower limbs progressed to develop angioinvasive mucormycosis. This further led to a thrombosis of the right external iliac artery and vein and rapidly progressive necrosis of surrounding soft tissues. She also had dextrocardia and patent foramen ovale. A right hip disarticulation and serial aggressive debridements were performed but she went on to develop systemic sepsis with multisystem involvement and succumbed to the infection. Pathology revealed mucor species with extensive vascular invasion.
CONCLUSION
This case highlights the importance of maintaining vigilance for mycotic infections and acting appropriately when there are signs of fulminant wound infection.
PubMed: 38633476
DOI: 10.5492/wjccm.v13.i1.86866 -
Texas Heart Institute Journal Apr 2024Dextrocardia with situs inversus totalis is a rare hereditary condition characterized by reversed orientation of the major thoracic and abdominal organs. Though...
Dextrocardia with situs inversus totalis is a rare hereditary condition characterized by reversed orientation of the major thoracic and abdominal organs. Though dextrocardia itself is not believed to increase the risk of coronary artery disease, the workup and surgical management of patients with this condition may be technically challenging to heart team clinicians. This report describes the case management of a high-risk 56-year-old man with dextrocardia who presented with multivessel coronary artery disease.
Topics: Male; Humans; Middle Aged; Coronary Artery Disease; Situs Inversus; Dextrocardia; Coronary Artery Bypass; Rare Diseases
PubMed: 38623731
DOI: 10.14503/THIJ-23-8382