outcome of aortic disease in these patients. The aim of this study was to investigate coagulation... more outcome of aortic disease in these patients. The aim of this study was to investigate coagulation disorders in a prospective Marfan cohort. Methods: Blood samples were collected in consecutive patients with verified Marfan syndrome who attended our outpatient clinic from December 2012 to October 2013. We analysed whole-blood platelet aggregation, aPTT, fibrinogen, D-dimers, Prothrombinfragment 1þ2 (F1þ2) and calibrated automated thrombogram (CAT). Results: 38 consecutive patients with proven Marfan syndrome (50% women, mean age 37.4 AE 11.5 years) were enrolled. Pathologic D-dimer levels were statistically significant in 5 patients, all of them with chronic dissection of the thoracic or abdominal aorta (p¼ 0.001, respectively). Fibrinogen was increased in patients with aortic regurgitation (p¼ 0.03) and there was a positive, significant correlation between age and fibrinogen (R¼ 0.43, p¼ 0.006). In vitro bleeding times (PFA-ADP-Collagen and PFA-Epinephrin-Collagen induced platelet function) were prolonged in 11 patients (28.9%, p¼ 0.009), which is compatible with von Willebrand syndrome or congenital thrombocytopathy. In addition, F1þ2 was elevated in 9 patients, whereas CAT results were decreased mostly. Conclusions: Coagulation plays a major role in Marfan syndrome. Both procoagulation and bleeding disorders participate in the disease. Platelet function abnormalities, compatible with von Willebrand syndrome or congenital thrombocytopathies, have been identified in 29% of patients. The results of different thrombin generation methods deserve further evaluation. As surgical procedures are often necessary in patients with marfan syndrome, the assessment of the coagulation status should be given greater attention.
Background: Arrhythmias are a well known complication in patients with single ventricle physiolog... more Background: Arrhythmias are a well known complication in patients with single ventricle physiology (SVP). However, there is still a lack of data regarding arrhythmias in older patients. The aim of this study was to analyze arrhythmia type and frequency, treatment and recurrence rates in patients with SVP over the age of 40 years. Methods: Data was obtained retrospectively from clinical records. All patients > 40 years with SVP with arrhythmias between 2005 and 2018 were included in the study. Treatment was classified as medical, interventional (electrophysiological studies (EPS) in combination with catheter ablation) or direct current cardioversion (DCCV). Results: Altogether, 29 patients (11 female; mean 47.5 ± 4.6 years) with 85 arrhythmia episodes were identified. The median follow-up time was 6.3 years. Cavo-tricuspid (CTI) and non-CTI related intra-atrial reentrant tachycardia (IART) and atrial fibrillation (AF) were most common (48.2% and 37.6%, respectively). In total, 18 ...
Background: There is an increasing number of adults with complete atrioventricular septal defects... more Background: There is an increasing number of adults with complete atrioventricular septal defects (cAVSD). However, data regarding older adults are lacking. The aim of this study is to analyze the outcome of adults with cAVSD over the age of 40 years. Methods: Patients with cAVSD who were ≥40 years of age at any point between 2005 until 2018 were included retrospectively. Data were retrieved from hospital records. The primary endpoint was a combination of death from any cause and unplanned hospitalizations due to cardiac reasons. Results: 43 patients (60.5% female, mean age 43.7 ± 6.0 years, genetic syndrome 58.1%) were included. At begin of follow-up, the majority of patients (n = 41, 95.3%) was in New York Heart Association (NYHA) class I or II. Out of the whole cohort 26 (60.5%) had undergone cardiac surgery. At baseline, at least one extracardiac comorbidity was present in 40 patients (93.0%). Median follow-up was 1.7 years (IQR 0.3–4.6). On univariate Cox analysis, NYHA class a...
Die pulmonal-arterielle Hypertonie (PAH) ist nach wie vor eine Erkrankung mit bedeutsamer Morbidi... more Die pulmonal-arterielle Hypertonie (PAH) ist nach wie vor eine Erkrankung mit bedeutsamer Morbiditat und Letalitat und ist haufig mit angeborenen Herzfehlern (AHF) assoziiert. Die Pravalenz der PAH-AHF ist in den entwickelten Landern in den vergangenen Jahren gefallen. Allerdings hat die Zahl der Erwachsenen mit PAH-AHF, die man heutzutage mehrheitlich in der klinischen Praxis mit komplexen oder spat diagnostizierten AHF trifft, deutlich zugenommen. In der Regel liegt einer PAH-AHF ein primarer links-rechts-Shunt zu Grunde. Durch den gesteigerten pulmonalen Blutfluss und zum Teil in Verbindung mit einem zusatzlich erhohten Druck in den Pulmonalarterien kommt es bei erhohtem Scherstress und vermehrter Wanddehnung zu einer endothelialen Dysfunktion und einem zunehmenden Remodeling der Lungenarterien und -arteriolen. Konsekutiv resultiert ein erhohter pulmonalvaskularer Widerstand. Die fortgeschrittenste Form der PAH-AHF ist das Eisenmenger-Syndrom, welches durch eine Shuntumkehr, d. h...
Fehlbildungen des Herzens und der herznahen Gefase (AHF) gehoren zu den haufigsten angeborenen An... more Fehlbildungen des Herzens und der herznahen Gefase (AHF) gehoren zu den haufigsten angeborenen Anomalien. Auf Grund des verbesserten medizinischen Managements erreichen etwa 90 % der Betroffenen behandelt oder unbehandelt das Erwachsenenalter. Bei bis zu 10 % dieser Patienten besteht oder entwickelt sich eine pulmonal-arterielle Hypertonie (PAH), die Belastungsfahigkeit und Prognose entscheidend beeintrachtigt. Gemas der 3. Weltkonferenz von 2008 in Dana Point wird die PAH bei AHF der Gruppe 1 der pulmonal-arteriellen Hypertonie zugeordnet. Anderungen dieser Klassifizierung wurden auf der Weltkonferenz in Nizza 2013 vorgeschlagen, sind aber noch nicht umgesetzt worden. Waren bis vor kurzer Zeit insbesondere die schwersten Formen der PAH bei AHF kaum behandelbar, so hat sich dies seit Einfuhrung der Endothelinrezeptor-Antagonisten, Phosphodiesterase-5-Inhibitoren und Prostanoide grundlegend geandert. Diese Substanzen sind in der Lage, Lebensqualitat und Prognose von Patienten mit PAH...
This author takes responsibility for all aspects of the reliability and freedom from bias of the ... more This author takes responsibility for all aspects of the reliability and freedom from bias of the data presented and their discussed interpretation.
n 11-year-old girl was admitted to a local hospital with syncope after gastroenteritis. During th... more n 11-year-old girl was admitted to a local hospital with syncope after gastroenteritis. During the work-up for syncope, a large tumor filling the left atrium was seen on transthoracic echocardiography. With the exemption of asthma, her past medical history was unremarkable, especially for cardiac symptoms. The patient was referred to our hospital. At presentation, she complained about recurrent shortness of breath, which was responsive to her asthma medication, and recurrent angina during the last few months. Physical examination and ECG revealed no abnormalities. The NT-proBNP (N-terminal pro-brain natriuretic peptide) was elevated (780 ng/L). Echocardiography showed a nonhomogeneous mass with a maximum size of 4×6 cm almost filling the left atrium completely and prolapsing through the mitral valve into the left ventricle during diastole (Figure [A], Movie I in the Data Supplement). The mitral valve ring was dilated, with moderate to severe mitral regurgitation and a mean gradient of 7 to 8 mm Hg. Cardiac magnetic resonance imaging confirmed a 61×32 mm measuring tumor, filling 80% of the atrial volume and prolapsing into the left ventricle during diastole (Figure [B]). It was isointense in T1-weighted and hyperintense in T2-weighted sequences and showed no contrast enhancement in perfusion sequences and heterogeneous late gadolinium enhancement. The size and function of the ventricles were normal. The decision for surgical excision was made. By an approach using median sternotomy and extracorporeal circulation, the left atrium was carefully opened, exposing a large tumor, which macroscopically resembled a myxoma (Figure [C]). In spite of the typical rather gelatinous and fragile consistency, the tumor could be removed in total including the pedicle. An annuloplasty (Medtronic Profile 3D 28 mm) was necessary because of severe mitral regurgitation. The postoperative course was uneventful, and the patient could be discharged with minimal mitral regurgitation (Movie II in the Data Supplement). Pathology confirmed the diagnosis of cardiac myxoma (Figure [D]). In children, primary cardiac tumors are rare with an incidence rate of 0.027% to 0.08% in autopsies. 1 Around 82% to 90% of primary cardiac tumors in children are benign. 1 Whereas in adults, myxoma is the most common primary cardiac tumor, in children, rhabdomyomas are most frequently encountered (50.7% of the benign cardiac tumors in children). Myxomas are also common (21.5% of the benign cardiac tumors in children). 1 The present case is unusual because of the size of the tumor. The symptoms of cardiac myxomas vary widely and depend on the size, location, and mobility of the tumor. Symptoms like shortness of breath, syncope, embolic events, congestive heart failure, and arrhythmias are often encountered. 2 Angina pectoris, which our patient reported, has also been described. 3 Echocardiography is the initial imaging modality for myxomas because its appearance is highly suggestive of the diagnosis: it is usually nonhomogenous with small lucencies or calcifications on it and localized at the atrial septum and has a pedicle. In contrast, an intracardiac
Eisenmenger syndrome as a severe form of cyanotic congenital heart disease results in a complex m... more Eisenmenger syndrome as a severe form of cyanotic congenital heart disease results in a complex multisystemic disorder. Due to increased systemic venous pressure and the inability to ensure systemic perfusion and metabolic requirements, the liver may develop congestion, fibrosis or cirrhosis. This study aimed to assess hepatic abnormalities in Eisenmenger patients non-invasively. 10 adults with Eisenmenger syndrome (six female; median age 44.2years; range 23-62years) were enrolled and hepatic involvement was assessed - using clinical assessment, laboratory analysis, hepatic fibrotic markers, abdominal sonography and liver stiffness measurements (transient elastography (TE) and acoustic radiation force impulse imaging (ARFI)). Using imaging and laboratory analysis, 60% (6/10) of the Eisenmenger patients had signs of liver fibrosis (5/10) or cirrhosis (1/10). While TE, however, showed no relevant liver abnormalities in any Eisenmenger patient, ARFI detected liver fibrosis in 5/10 and ...
At the present time there is still concern regarding the long-term deleterious effects of right v... more At the present time there is still concern regarding the long-term deleterious effects of right ventricular apical pacing in patients referred for auriculoventricular node ablation (AVNA). Furthermore, scarce information is available regarding differences in the follow up according to the baseline cardiopathy and predictors associated with a worse outcome. 104 consecutives patients referred for AVNA were retrospectively analyzed. Patients included were seen in the outpatient clinic at 6, 12 and 24 months post ablation (mean follow-up 24 ± 2 months). An echocardiogram two years after the procedure was obtained in 68 patients. Three categories were done according to the change in the left ventricular function (LVEF) (increase, decrease or absence of change, defined as less than 10% variation in either LVEF). After two years of follow up there was a decrease in the rate of hospital admission (from 0.9 admission/year to 0.35, p<0.001), an increase in the functional status in at least...
Erfolgreiche chirurgische Verfahren, interventionelle Prozeduren und neue medikamentöse Therapien... more Erfolgreiche chirurgische Verfahren, interventionelle Prozeduren und neue medikamentöse Therapien haben die Überlebenswahrscheinlichkeiten von Patienten mit angeborenen Herzfehlern (AHF) in den letzten Jahrzehnten drastisch verbessert. Bei ebenfalls steigender Lebensqualität der betroffenen Frauen steigt deren Kinderwunsch und es erwächst ein zunehmender Informationsbedarf hinsichtlich einer adäquaten Schwangerschaftsführung. Frauen mit AHF können unter adäquater kardiologischer und geburtshilflicher Führung häufig auch mehrere Schwangerschaften und Geburten bewältigen. Nur bei wenigen Herzfehlern besteht durch eine Schwangerschaft ein so hohes Risiko, dass von einer Schwangerschaft abgeraten bzw. eine Schwangerschaft abgebrochen werden sollte. In Deutschland schätzt man die Zahl von Erwachsenen mit AHF auf bis zu 280 000 und erwartet eine Zunahme um etwa 5000 Patienten pro Jahr [1, 2]. Eine kürzlich publizierte Studie aus den USA [3] beschrieb einen Anteil von 2% an kardialen Erkrankungen bei schwangeren Frauen, wobei hier die angeborenen Herzfehler den größten Teil ausmachen, da die rheumatischen Erkrankungen bei uns eine zunehmend geringere Rolle spielen. Geburtshelfer und Gynäkologen werden hier zunehmend mit dieser Patientengruppe konfrontiert werden. Umstellungsreaktionen im Zuge der Schwangerschaft Unmittelbar mit der Konzeption beginnen die Umstellungsreaktionen des mütterlichen Kreislaufsystems, die v. a. folgende Parameter umfassen:
outcome of aortic disease in these patients. The aim of this study was to investigate coagulation... more outcome of aortic disease in these patients. The aim of this study was to investigate coagulation disorders in a prospective Marfan cohort. Methods: Blood samples were collected in consecutive patients with verified Marfan syndrome who attended our outpatient clinic from December 2012 to October 2013. We analysed whole-blood platelet aggregation, aPTT, fibrinogen, D-dimers, Prothrombinfragment 1þ2 (F1þ2) and calibrated automated thrombogram (CAT). Results: 38 consecutive patients with proven Marfan syndrome (50% women, mean age 37.4 AE 11.5 years) were enrolled. Pathologic D-dimer levels were statistically significant in 5 patients, all of them with chronic dissection of the thoracic or abdominal aorta (p¼ 0.001, respectively). Fibrinogen was increased in patients with aortic regurgitation (p¼ 0.03) and there was a positive, significant correlation between age and fibrinogen (R¼ 0.43, p¼ 0.006). In vitro bleeding times (PFA-ADP-Collagen and PFA-Epinephrin-Collagen induced platelet function) were prolonged in 11 patients (28.9%, p¼ 0.009), which is compatible with von Willebrand syndrome or congenital thrombocytopathy. In addition, F1þ2 was elevated in 9 patients, whereas CAT results were decreased mostly. Conclusions: Coagulation plays a major role in Marfan syndrome. Both procoagulation and bleeding disorders participate in the disease. Platelet function abnormalities, compatible with von Willebrand syndrome or congenital thrombocytopathies, have been identified in 29% of patients. The results of different thrombin generation methods deserve further evaluation. As surgical procedures are often necessary in patients with marfan syndrome, the assessment of the coagulation status should be given greater attention.
As thromboembolic events (TEE) are common in adults with congenital heart disease (ACHD), adequat... more As thromboembolic events (TEE) are common in adults with congenital heart disease (ACHD), adequate oral anticoagulation for prophylaxis or treatment of TEE is important. Until now, mainly vitamin K antagonists have been used in these patients. The purpose of this study was to provide first data on the use of direct oral anticoagulants in ACHD. This prospective, observational, and longitudinal study included 102 consecutive ACHD, of whom 75 (37 women and 38 men; mean age 50-13 years) could be analyzed. Most common CHD were pre-tricuspid shunts (n [ 31; 41%), complex CHD (n [ 16; 21%), left heart/aortic valve anomalies (n [ 5; 6%), right-sided cardiac/pulmonary artery anomalies (n [ 9; 13%), posttricuspid shunts (n [ 3; 4%), and others (n [ 11; 15%). Five patients had cyanosis and 3 patients a Fontan circulation. Mean follow-up was 12-11 months. Rivaroxaban was administered in 55 patients, apixaban in 13 and dabigatran in 7 patients for TEE prophylaxis in atrial arrhythmias (n [ 57), stroke/transient ischemic attacks (n [ 11), deep vein thrombosis (n [ 4), pulmonary embolism (n [ 1) and atrial thrombi (n [ 3). Some patients had >1 indication for adequate oral anticoagulation. CHA 2-DS 2-VASc score was ‡2 in 23 (31%), and 9 (12%) had a HAS-BLED score ‡2. There were neither thrombotic or major bleeding events nor major side effects. In conclusion, direct oral anticoagulants appear to be safe and effective in ACHD. Long-term follow-up is needed to substantiate these findings. Ó 2016 Elsevier Inc. All rights reserved. (Am J Cardiol 2016;117:450e455) Cardiovascular disorders, including thromboembolic events (TEE), are the most common causes of death. As many adults with congenital heart disease (ACHD) are prone to TEE, and morbidity and mortality from these complications are also high, adequate oral anticoagulation (OAC) for prophylaxis or treatment of TEE is of outstanding importance. To date, only data about the use of vitamin K antagonists (VKA) are available in these patients. 1 Data on the use of novel or direct oral anticoagulants (DOAC) in ACHD are scarce. 2 Therefore, the purpose of this analysis is to provide first real-world experience regarding the anticoagulation management of ACHD using DOACs.
outcome of aortic disease in these patients. The aim of this study was to investigate coagulation... more outcome of aortic disease in these patients. The aim of this study was to investigate coagulation disorders in a prospective Marfan cohort. Methods: Blood samples were collected in consecutive patients with verified Marfan syndrome who attended our outpatient clinic from December 2012 to October 2013. We analysed whole-blood platelet aggregation, aPTT, fibrinogen, D-dimers, Prothrombinfragment 1þ2 (F1þ2) and calibrated automated thrombogram (CAT). Results: 38 consecutive patients with proven Marfan syndrome (50% women, mean age 37.4 AE 11.5 years) were enrolled. Pathologic D-dimer levels were statistically significant in 5 patients, all of them with chronic dissection of the thoracic or abdominal aorta (p¼ 0.001, respectively). Fibrinogen was increased in patients with aortic regurgitation (p¼ 0.03) and there was a positive, significant correlation between age and fibrinogen (R¼ 0.43, p¼ 0.006). In vitro bleeding times (PFA-ADP-Collagen and PFA-Epinephrin-Collagen induced platelet function) were prolonged in 11 patients (28.9%, p¼ 0.009), which is compatible with von Willebrand syndrome or congenital thrombocytopathy. In addition, F1þ2 was elevated in 9 patients, whereas CAT results were decreased mostly. Conclusions: Coagulation plays a major role in Marfan syndrome. Both procoagulation and bleeding disorders participate in the disease. Platelet function abnormalities, compatible with von Willebrand syndrome or congenital thrombocytopathies, have been identified in 29% of patients. The results of different thrombin generation methods deserve further evaluation. As surgical procedures are often necessary in patients with marfan syndrome, the assessment of the coagulation status should be given greater attention.
Background: Arrhythmias are a well known complication in patients with single ventricle physiolog... more Background: Arrhythmias are a well known complication in patients with single ventricle physiology (SVP). However, there is still a lack of data regarding arrhythmias in older patients. The aim of this study was to analyze arrhythmia type and frequency, treatment and recurrence rates in patients with SVP over the age of 40 years. Methods: Data was obtained retrospectively from clinical records. All patients > 40 years with SVP with arrhythmias between 2005 and 2018 were included in the study. Treatment was classified as medical, interventional (electrophysiological studies (EPS) in combination with catheter ablation) or direct current cardioversion (DCCV). Results: Altogether, 29 patients (11 female; mean 47.5 ± 4.6 years) with 85 arrhythmia episodes were identified. The median follow-up time was 6.3 years. Cavo-tricuspid (CTI) and non-CTI related intra-atrial reentrant tachycardia (IART) and atrial fibrillation (AF) were most common (48.2% and 37.6%, respectively). In total, 18 ...
Background: There is an increasing number of adults with complete atrioventricular septal defects... more Background: There is an increasing number of adults with complete atrioventricular septal defects (cAVSD). However, data regarding older adults are lacking. The aim of this study is to analyze the outcome of adults with cAVSD over the age of 40 years. Methods: Patients with cAVSD who were ≥40 years of age at any point between 2005 until 2018 were included retrospectively. Data were retrieved from hospital records. The primary endpoint was a combination of death from any cause and unplanned hospitalizations due to cardiac reasons. Results: 43 patients (60.5% female, mean age 43.7 ± 6.0 years, genetic syndrome 58.1%) were included. At begin of follow-up, the majority of patients (n = 41, 95.3%) was in New York Heart Association (NYHA) class I or II. Out of the whole cohort 26 (60.5%) had undergone cardiac surgery. At baseline, at least one extracardiac comorbidity was present in 40 patients (93.0%). Median follow-up was 1.7 years (IQR 0.3–4.6). On univariate Cox analysis, NYHA class a...
Die pulmonal-arterielle Hypertonie (PAH) ist nach wie vor eine Erkrankung mit bedeutsamer Morbidi... more Die pulmonal-arterielle Hypertonie (PAH) ist nach wie vor eine Erkrankung mit bedeutsamer Morbiditat und Letalitat und ist haufig mit angeborenen Herzfehlern (AHF) assoziiert. Die Pravalenz der PAH-AHF ist in den entwickelten Landern in den vergangenen Jahren gefallen. Allerdings hat die Zahl der Erwachsenen mit PAH-AHF, die man heutzutage mehrheitlich in der klinischen Praxis mit komplexen oder spat diagnostizierten AHF trifft, deutlich zugenommen. In der Regel liegt einer PAH-AHF ein primarer links-rechts-Shunt zu Grunde. Durch den gesteigerten pulmonalen Blutfluss und zum Teil in Verbindung mit einem zusatzlich erhohten Druck in den Pulmonalarterien kommt es bei erhohtem Scherstress und vermehrter Wanddehnung zu einer endothelialen Dysfunktion und einem zunehmenden Remodeling der Lungenarterien und -arteriolen. Konsekutiv resultiert ein erhohter pulmonalvaskularer Widerstand. Die fortgeschrittenste Form der PAH-AHF ist das Eisenmenger-Syndrom, welches durch eine Shuntumkehr, d. h...
Fehlbildungen des Herzens und der herznahen Gefase (AHF) gehoren zu den haufigsten angeborenen An... more Fehlbildungen des Herzens und der herznahen Gefase (AHF) gehoren zu den haufigsten angeborenen Anomalien. Auf Grund des verbesserten medizinischen Managements erreichen etwa 90 % der Betroffenen behandelt oder unbehandelt das Erwachsenenalter. Bei bis zu 10 % dieser Patienten besteht oder entwickelt sich eine pulmonal-arterielle Hypertonie (PAH), die Belastungsfahigkeit und Prognose entscheidend beeintrachtigt. Gemas der 3. Weltkonferenz von 2008 in Dana Point wird die PAH bei AHF der Gruppe 1 der pulmonal-arteriellen Hypertonie zugeordnet. Anderungen dieser Klassifizierung wurden auf der Weltkonferenz in Nizza 2013 vorgeschlagen, sind aber noch nicht umgesetzt worden. Waren bis vor kurzer Zeit insbesondere die schwersten Formen der PAH bei AHF kaum behandelbar, so hat sich dies seit Einfuhrung der Endothelinrezeptor-Antagonisten, Phosphodiesterase-5-Inhibitoren und Prostanoide grundlegend geandert. Diese Substanzen sind in der Lage, Lebensqualitat und Prognose von Patienten mit PAH...
This author takes responsibility for all aspects of the reliability and freedom from bias of the ... more This author takes responsibility for all aspects of the reliability and freedom from bias of the data presented and their discussed interpretation.
n 11-year-old girl was admitted to a local hospital with syncope after gastroenteritis. During th... more n 11-year-old girl was admitted to a local hospital with syncope after gastroenteritis. During the work-up for syncope, a large tumor filling the left atrium was seen on transthoracic echocardiography. With the exemption of asthma, her past medical history was unremarkable, especially for cardiac symptoms. The patient was referred to our hospital. At presentation, she complained about recurrent shortness of breath, which was responsive to her asthma medication, and recurrent angina during the last few months. Physical examination and ECG revealed no abnormalities. The NT-proBNP (N-terminal pro-brain natriuretic peptide) was elevated (780 ng/L). Echocardiography showed a nonhomogeneous mass with a maximum size of 4×6 cm almost filling the left atrium completely and prolapsing through the mitral valve into the left ventricle during diastole (Figure [A], Movie I in the Data Supplement). The mitral valve ring was dilated, with moderate to severe mitral regurgitation and a mean gradient of 7 to 8 mm Hg. Cardiac magnetic resonance imaging confirmed a 61×32 mm measuring tumor, filling 80% of the atrial volume and prolapsing into the left ventricle during diastole (Figure [B]). It was isointense in T1-weighted and hyperintense in T2-weighted sequences and showed no contrast enhancement in perfusion sequences and heterogeneous late gadolinium enhancement. The size and function of the ventricles were normal. The decision for surgical excision was made. By an approach using median sternotomy and extracorporeal circulation, the left atrium was carefully opened, exposing a large tumor, which macroscopically resembled a myxoma (Figure [C]). In spite of the typical rather gelatinous and fragile consistency, the tumor could be removed in total including the pedicle. An annuloplasty (Medtronic Profile 3D 28 mm) was necessary because of severe mitral regurgitation. The postoperative course was uneventful, and the patient could be discharged with minimal mitral regurgitation (Movie II in the Data Supplement). Pathology confirmed the diagnosis of cardiac myxoma (Figure [D]). In children, primary cardiac tumors are rare with an incidence rate of 0.027% to 0.08% in autopsies. 1 Around 82% to 90% of primary cardiac tumors in children are benign. 1 Whereas in adults, myxoma is the most common primary cardiac tumor, in children, rhabdomyomas are most frequently encountered (50.7% of the benign cardiac tumors in children). Myxomas are also common (21.5% of the benign cardiac tumors in children). 1 The present case is unusual because of the size of the tumor. The symptoms of cardiac myxomas vary widely and depend on the size, location, and mobility of the tumor. Symptoms like shortness of breath, syncope, embolic events, congestive heart failure, and arrhythmias are often encountered. 2 Angina pectoris, which our patient reported, has also been described. 3 Echocardiography is the initial imaging modality for myxomas because its appearance is highly suggestive of the diagnosis: it is usually nonhomogenous with small lucencies or calcifications on it and localized at the atrial septum and has a pedicle. In contrast, an intracardiac
Eisenmenger syndrome as a severe form of cyanotic congenital heart disease results in a complex m... more Eisenmenger syndrome as a severe form of cyanotic congenital heart disease results in a complex multisystemic disorder. Due to increased systemic venous pressure and the inability to ensure systemic perfusion and metabolic requirements, the liver may develop congestion, fibrosis or cirrhosis. This study aimed to assess hepatic abnormalities in Eisenmenger patients non-invasively. 10 adults with Eisenmenger syndrome (six female; median age 44.2years; range 23-62years) were enrolled and hepatic involvement was assessed - using clinical assessment, laboratory analysis, hepatic fibrotic markers, abdominal sonography and liver stiffness measurements (transient elastography (TE) and acoustic radiation force impulse imaging (ARFI)). Using imaging and laboratory analysis, 60% (6/10) of the Eisenmenger patients had signs of liver fibrosis (5/10) or cirrhosis (1/10). While TE, however, showed no relevant liver abnormalities in any Eisenmenger patient, ARFI detected liver fibrosis in 5/10 and ...
At the present time there is still concern regarding the long-term deleterious effects of right v... more At the present time there is still concern regarding the long-term deleterious effects of right ventricular apical pacing in patients referred for auriculoventricular node ablation (AVNA). Furthermore, scarce information is available regarding differences in the follow up according to the baseline cardiopathy and predictors associated with a worse outcome. 104 consecutives patients referred for AVNA were retrospectively analyzed. Patients included were seen in the outpatient clinic at 6, 12 and 24 months post ablation (mean follow-up 24 ± 2 months). An echocardiogram two years after the procedure was obtained in 68 patients. Three categories were done according to the change in the left ventricular function (LVEF) (increase, decrease or absence of change, defined as less than 10% variation in either LVEF). After two years of follow up there was a decrease in the rate of hospital admission (from 0.9 admission/year to 0.35, p<0.001), an increase in the functional status in at least...
Erfolgreiche chirurgische Verfahren, interventionelle Prozeduren und neue medikamentöse Therapien... more Erfolgreiche chirurgische Verfahren, interventionelle Prozeduren und neue medikamentöse Therapien haben die Überlebenswahrscheinlichkeiten von Patienten mit angeborenen Herzfehlern (AHF) in den letzten Jahrzehnten drastisch verbessert. Bei ebenfalls steigender Lebensqualität der betroffenen Frauen steigt deren Kinderwunsch und es erwächst ein zunehmender Informationsbedarf hinsichtlich einer adäquaten Schwangerschaftsführung. Frauen mit AHF können unter adäquater kardiologischer und geburtshilflicher Führung häufig auch mehrere Schwangerschaften und Geburten bewältigen. Nur bei wenigen Herzfehlern besteht durch eine Schwangerschaft ein so hohes Risiko, dass von einer Schwangerschaft abgeraten bzw. eine Schwangerschaft abgebrochen werden sollte. In Deutschland schätzt man die Zahl von Erwachsenen mit AHF auf bis zu 280 000 und erwartet eine Zunahme um etwa 5000 Patienten pro Jahr [1, 2]. Eine kürzlich publizierte Studie aus den USA [3] beschrieb einen Anteil von 2% an kardialen Erkrankungen bei schwangeren Frauen, wobei hier die angeborenen Herzfehler den größten Teil ausmachen, da die rheumatischen Erkrankungen bei uns eine zunehmend geringere Rolle spielen. Geburtshelfer und Gynäkologen werden hier zunehmend mit dieser Patientengruppe konfrontiert werden. Umstellungsreaktionen im Zuge der Schwangerschaft Unmittelbar mit der Konzeption beginnen die Umstellungsreaktionen des mütterlichen Kreislaufsystems, die v. a. folgende Parameter umfassen:
outcome of aortic disease in these patients. The aim of this study was to investigate coagulation... more outcome of aortic disease in these patients. The aim of this study was to investigate coagulation disorders in a prospective Marfan cohort. Methods: Blood samples were collected in consecutive patients with verified Marfan syndrome who attended our outpatient clinic from December 2012 to October 2013. We analysed whole-blood platelet aggregation, aPTT, fibrinogen, D-dimers, Prothrombinfragment 1þ2 (F1þ2) and calibrated automated thrombogram (CAT). Results: 38 consecutive patients with proven Marfan syndrome (50% women, mean age 37.4 AE 11.5 years) were enrolled. Pathologic D-dimer levels were statistically significant in 5 patients, all of them with chronic dissection of the thoracic or abdominal aorta (p¼ 0.001, respectively). Fibrinogen was increased in patients with aortic regurgitation (p¼ 0.03) and there was a positive, significant correlation between age and fibrinogen (R¼ 0.43, p¼ 0.006). In vitro bleeding times (PFA-ADP-Collagen and PFA-Epinephrin-Collagen induced platelet function) were prolonged in 11 patients (28.9%, p¼ 0.009), which is compatible with von Willebrand syndrome or congenital thrombocytopathy. In addition, F1þ2 was elevated in 9 patients, whereas CAT results were decreased mostly. Conclusions: Coagulation plays a major role in Marfan syndrome. Both procoagulation and bleeding disorders participate in the disease. Platelet function abnormalities, compatible with von Willebrand syndrome or congenital thrombocytopathies, have been identified in 29% of patients. The results of different thrombin generation methods deserve further evaluation. As surgical procedures are often necessary in patients with marfan syndrome, the assessment of the coagulation status should be given greater attention.
As thromboembolic events (TEE) are common in adults with congenital heart disease (ACHD), adequat... more As thromboembolic events (TEE) are common in adults with congenital heart disease (ACHD), adequate oral anticoagulation for prophylaxis or treatment of TEE is important. Until now, mainly vitamin K antagonists have been used in these patients. The purpose of this study was to provide first data on the use of direct oral anticoagulants in ACHD. This prospective, observational, and longitudinal study included 102 consecutive ACHD, of whom 75 (37 women and 38 men; mean age 50-13 years) could be analyzed. Most common CHD were pre-tricuspid shunts (n [ 31; 41%), complex CHD (n [ 16; 21%), left heart/aortic valve anomalies (n [ 5; 6%), right-sided cardiac/pulmonary artery anomalies (n [ 9; 13%), posttricuspid shunts (n [ 3; 4%), and others (n [ 11; 15%). Five patients had cyanosis and 3 patients a Fontan circulation. Mean follow-up was 12-11 months. Rivaroxaban was administered in 55 patients, apixaban in 13 and dabigatran in 7 patients for TEE prophylaxis in atrial arrhythmias (n [ 57), stroke/transient ischemic attacks (n [ 11), deep vein thrombosis (n [ 4), pulmonary embolism (n [ 1) and atrial thrombi (n [ 3). Some patients had >1 indication for adequate oral anticoagulation. CHA 2-DS 2-VASc score was ‡2 in 23 (31%), and 9 (12%) had a HAS-BLED score ‡2. There were neither thrombotic or major bleeding events nor major side effects. In conclusion, direct oral anticoagulants appear to be safe and effective in ACHD. Long-term follow-up is needed to substantiate these findings. Ó 2016 Elsevier Inc. All rights reserved. (Am J Cardiol 2016;117:450e455) Cardiovascular disorders, including thromboembolic events (TEE), are the most common causes of death. As many adults with congenital heart disease (ACHD) are prone to TEE, and morbidity and mortality from these complications are also high, adequate oral anticoagulation (OAC) for prophylaxis or treatment of TEE is of outstanding importance. To date, only data about the use of vitamin K antagonists (VKA) are available in these patients. 1 Data on the use of novel or direct oral anticoagulants (DOAC) in ACHD are scarce. 2 Therefore, the purpose of this analysis is to provide first real-world experience regarding the anticoagulation management of ACHD using DOACs.
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