Background Cardiac autonomic dysfunction after myocardial infarction identifies patients at high risk despite only moderately reduced left ventricular ejection fraction. We aimed to show that telemedical monitoring with implantable cardiac monitors in these patients can improve early detection of subclinical but prognostically relevant arrhythmic events. Methods We did a prospective investigator-initiated, randomised, multicentre, open-label, diagnostic trial at 33 centres in Germany and Austria. Survivors of acute myocardial infarction with left ventricular ejection fraction of 36-50% had biosignal analysis for assessment of cardiac autonoinic function. Patients with abnormal periodic repolarisation dynamics (>= 5.75 deg(2)) or abnormal deceleration capacity (<= 2.5 ms) were randomly assigned (1:1) to telemedical monitoring with implantable cardiac monitors or conventional follow-up. Primary endpoint was time to detection of serious arrhythmic events defined by atrial fibrillation 6 min or longer, atrioventricular block class IIb or higher and fast non-sustained (>187 beats per min; >= 40 beats) or sustained ventricular tachycardia or fibrillation. This study is registered with ClinicalTrials.gov, NCT02594488. Findings Between May 12, 2016, and July 20, 2020, 1305 individuals were screened and 400 patients at high risk were randomly assigned (median age 64 years [IQR 57-73]); left ventricular ejection fraction 45% [40-48]) to telemedical monitoring with implantable cardiac monitors (implantable cardiac monitor group; n=201) or conventional follow-up (control group; n=199). During median follow-up of 21 months, serious arrhythmic events were detected in 60 (30%) patients of the implantable cardiac monitor group and 12 (6%) patients of the control group (hazard ratio 6.33 [IQR 3.40-11.78]; p<0.001). An improved detection rate by implantable cardiac monitors was observed for all types of serious arrhythmic events: atrial fibrillation 6 min or longer (47 [23%] patients vs 11 [16%] patients; p<0.001), atrioventricular block class IIb or higher (14 [7%] vs 0; p<0.001) and ventricular tachycardia or ventricular fibrillation (nine [4%] patients vs two [1%] patients; p=0.054). Interpretation In patients at high risk after myocardial infarction and cardiac autonomic dysfunction but only moderately reduced left ventricular ejection fraction, telemedical monitoring with implantable cardiac monitors was highly effective in early detection of subclinical, prognostically relevant serious arrhythmic events. Copyright (C) 2022 The Author(s). Published by Elsevier Ltd.

BACKGROUND:COVID-19 is an infectious disease characterized by various clinical presentations. Knowledge of possible symptoms and their distribution allows for the early identification of infected patients.OBJECTIVE:To determine the distribution pattern of COVID-19 symptoms as well as possible unreported symptoms, we created an app-based self-reporting tool.METHODS:The COVID-19 Symptom Tracker is an app-based daily self-reporting tool. Between April 8 and May 15, 2020, a total of 22,327 individuals installed this app on their mobile device. An initial questionnaire asked for demographic information (age, gender, postal code) and past medical history comprising relevant chronic diseases. The participants were reminded daily to report whether they were experiencing any symptoms and if they had been tested for SARS-CoV-2 infection. Participants who sought health care services were asked additional questions regarding diagnostics and treatment. Participation was open to all adults (≥18 years). The study was completely anonymous.RESULTS:In total, 11,829 (52.98%) participants completed the symptom questionnaire at least once. Of these, 291 (2.46%) participants stated that they had undergone an RT-PCR (reverse transcription-polymerase chain reaction) test for SARS-CoV-2; 65 (0.55%) reported a positive test result and 226 (1.91%) a negative one. The mean number of reported symptoms among untested participants was 0.81 (SD 1.85). Participants with a positive test result had, on average, 5.63 symptoms (SD 2.82). The most significant risk factors were diabetes (odds ratio [OR] 8.95, 95% CI 3.30-22.37) and chronic heart disease (OR 2.85, 95% CI 1.43-5.69). We identified chills, fever, loss of smell, nausea and vomiting, and shortness of breath as the top five strongest predictors for a COVID-19 infection. The odds ratio for loss of smell was 3.13 (95% CI 1.76-5.58). Nausea and vomiting (OR 2.84, 95% CI 1.61-5.00) had been reported as an uncommon symptom previously; however, our data suggest a significant predictive value.CONCLUSIONS:Self-reported symptom tracking helps to identify novel symptoms of COVID-19 and to estimate the predictive value of certain symptoms. This aids in the development of reliable screening tools. Clinical screening with a high pretest probability allows for the rapid identification of infections and the cost-effective use of testing resources. Based on our results, we suggest that loss of smell and taste be considered cardinal symptoms; we also stress that diabetes is a risk factor for a highly symptomatic course of COVID-19 infection.

<sec> &lt;title&gt;BACKGROUND&lt;/title&gt; &lt;p&gt;COVID-19 is an infection characterized by various different clinical presentations. Knowledge of possible symptoms and their distribution allows an early identification of infected patients.&lt;/p&gt; </sec> <sec> &lt;title&gt;OBJECTIVE&lt;/title&gt; &lt;p&gt;To determine the distribution pattern and possible unreported symptoms an app-based self-reporting tool was created.&lt;/p&gt; </sec> <sec> &lt;title&gt;METHODS&lt;/title&gt; &lt;p&gt;The COVID-19 Symptom Tracker study is an app-based daily self-reporting study. Between 08 April and 15 May 2020, a total of 22,327 individuals installed the smartphone app (COVID-19 Symptom Tracker) on their mobile device. An initial questionnaire asks for demographic information (age, gender, post code) and a past medical history with relevant chronic diseases. The participants are notified daily to report whether they are suffering from current symptoms and have been tested for SARS-CoV-2. When seeking healthcare advice additional questions regarding diagnostics and therapy are asked. Participation is open for every adult (minimum age 18 years). The study is completely anonymous.&lt;/p&gt; </sec> <sec> &lt;title&gt;RESULTS&lt;/title&gt; &lt;p&gt;11,829 (52.98%) participants completed the symptom questionnaire at least once. 291 of these participants stated that a RT-PCR test for SARS-CoV-2 was performed. 65 reported a positive and 226 a negative test result. The mean average number of reported symptoms in the group of untested participants was 0.81 (SD: 1.85). Participants with a positive test showed a mean average of 5.63 symptoms (SD: 2.82). Most significant risk factors are diabetes (OR: 8.95; CI: 3.30-22.37) and chronic heart disease (OR: 2.85; CI: 1.43-5.69). We identified chills, fever, loss of smell, nausea and vomiting and shortness of breath as the top five of the strongest predictors for a COVID-19 infection. The odds ratio (with 95% confidence interval) for loss of smell was 3.13 (1.76-5.58). Nausea and vomiting (OR: 2.84; CI: 1.61-5.00) has been reported as an uncommon symptom however our data suggest a significant predictive value.&lt;/p&gt; </sec> <sec> &lt;title&gt;CONCLUSIONS&lt;/title&gt; &lt;p&gt;Self-reported symptom tracking helps to identify novel symptoms of the COVID-19 disease and estimate the predictive value of certain symptoms. This helps to develop reliable screening tools. A clinical screening with a high pre-test probability allows the rapid identification of infections and a cost-effective use of testing resources. Our data stress the necessity for an awareness of loss of smell and taste as a cardinal symptom and suggest that diabetes is a risk factor for a highly symptomatic course of a COVID-19 infection.&lt;/p&gt; </sec> <sec> &lt;title&gt;CLINICALTRIAL&lt;/title&gt; &lt;p&gt;DNA&lt;/p&gt; </sec>

AIMS:Cardiac energy requirement is met to a large extent by oxidative phosphorylation in mitochondria that are highly abundant in cardiac myocytes. Human mitochondrial thioredoxin reductase (TXNRD2) is a selenocysteine-containing enzyme essential for mitochondrial oxygen radical scavenging. Cardiac-specific deletion of Txnrd2 in mice results in dilated cardiomyopathy (DCM). The aim of this study was to investigate whether TXNRD2 mutations explain a fraction of monogenic DCM cases.METHODS AND RESULTS:Sequencing and subsequent genotyping of TXNRD2 in patients diagnosed with DCM (n = 227) and in DCM-free (n = 683) individuals from the general population sample KORA S4 was performed. The functional impact of observed mutations on Txnrd2 function was tested in mouse fibroblasts. We identified two novel amino acid residue-altering TXNRD2 mutations [175G > A (Ala59Thr) and 1124G > A (Gly375Arg)] in three heterozygous carriers among 227 patients that were not observed in the 683 DCM-free individuals. Both DCM-associated mutations result in amino acid substitutions of highly conserved residues in helices contributing to the flavin-adenine dinucleotide (FAD)-binding domain of TXNRD2. Functional analysis of both mutations in Txnrd2(-/-) mouse fibroblasts revealed that contrasting to wild-type (wt) Txnrd2, neither mutant did restore Txnrd2 function. Mutants even impaired the survival of Txnrd2 wt cells under oxidative stress by a dominant-negative mechanism.CONCLUSION:For the first time, we describe mutations in DCM patients in a gene involved in the regulation of cellular redox state. TXNRD2 mutations may explain a fraction of human DCM disease burden.

Inappropriate sinus tachycardia (IST) is characterized by paroxysmal tachycardia originating in the sinus nodal area. IST predominately affects young, female patients. Current antiarrhythmic drug treatment (ß-blockers, calcium antagonists), frequently complicated by side effects, is often not successful. Ivabradine, approved for angina pectoris, selectively reduces heart rate by blocking the “funny current” in the sinus node. We therefore evaluated the effect of ivabradine in patients with symptomatic IST. Ten female patients (median age 32.5 years, range 12–57) suffering from symptomatic IST who had either failed (n = 8) or refused (n = 2) conventional therapy were analyzed. Symptoms included palpitations, pre-syncope, syncope, dyspnea, and exercise intolerance. After obtaining informed consent for individual off-label therapy, patients were treated with ivabradine (5–7.5 mg bid) in addition to beta-blocker therapy (n = 3) or as mono- therapy (n = 7). Therapy was monitored by 72-h Holter ECG and a symptoms questionnaire. Ivabradine significantly reduced maximum and mean heart rate (baseline, maximal heart rate 176 ± 45/min, mean heart rate 84 ± 11/min; ivabradine, maximal heart rate 137 ± 36/min, mean HR 74 ± 8/min, both p < 0.05, all values as mean ± SD). Minimum heart rate was not significantly changed. Three patients reported transient phosphene-like phenomena without discontinuation of ivabradine while on therapy. IST-associated symptoms were ameliorated (3 pts) or suppressed (5 pts) in all eight patients who could be contacted after a mean follow-up of 16 ± 9 months. Ivabradine appears effective and safe in patients with symptomatic inappropriate sinus tachycardia.

Development of a paravalvular leak after prosthetic valve replacement is a rare but hard to treat complication. In many cases, it requires reoperation. Reoperation is associated with higher mortality and excess risk of recurrent paravalvular insufficiency. During the last couple of years, several hybrid procedures have been developed to decrease operative risk and trauma.1Schmitz C. Esmailzadeh B. Herberg U. Lang N. Sodian R. Kozlik-Feldmann R. et al.Hybrid procedures can reduce the risk of congenital cardiovascular surgery.Eur J Cardiothorac Surg. 2008; 34: 718-725Crossref PubMed Scopus (30) Google Scholar We describe the case of a 66-year old man after replacement of the aortic and mitral valves (Medtronic Hall; Medtronic, Inc, Minneapolis, Minn) in 1989. In 2000, the ascending aorta was replaced, and a DDD pacer was implanted. In 2008, the patient had acute hemolysis, which necessitated periodic blood transfusions. Transthoracic echocardiography revealed a new paravalvular leak of the mitral valve (Figure 1). Because of multiple comorbidities (renal insufficiency, hypertension, previous hemicolectomy), we discussed with the patient the option of implanting an Amplatzer Septal Occluder in a hybrid fashion. With the patient under general anesthesia, the chest was opened through a small (8 cm) left anterolateral thoracotomy to present the left ventricular apex. The pericardium was incised just above the apex, and two Teflon-armed purse-string sutures (Ethicon, Johnson & Johnson, Norder Stedt, Germany) were placed on the left ventricular apex. A short 8F sheath (Cordis, Johnson & Johnson, Warren, NJ) was inserted through a stab wound incision into the left ventricle. A Terumo 0.025–inch guidewire (Termumo Medical Corporation, Somerset, NJ) was introduced under transesophageal echocardiographic and fluoroscopic guidance and pushed forward through the annular dehiscence into the left atrium. The sheath was advanced into the left atrium, and a 6-mm Membranous Amplatzer Septal Occluder (AGA Medical, Golden Valley, Minn) was implanted. After sheath removal, the purse-string sutures were tied, and the chest was closed in layers. Overall skin-to-skin operative time was 140 minutes. The patient was weaned from the ventilator on postoperative day 1, was transferred from the intensive care unit on postoperative day 2, and was discharged home on postoperative day published 23. During the hospital stay, the pacemaker was replaced. Transthoracic echocardiography showed no residual leakage. Function of both valve prostheses was normal. There have been several reports on closing paravalvular leaks with different umbrella-type devices.2Sivakumar K. Shahani J. Transcatheter closure of paravalvular mitral prosthetic leak with resultant hemolysis.Int J Cardiol. 2007; 115: e39-e40Abstract Full Text Full Text PDF PubMed Scopus (21) Google Scholar, 3Webb J.G. Pate G.E. Munt B.I. Percutaneous closure of an aortic prosthetic paravalvular leak with an Amplatzer duct occluder.Catheter Cardiovasc Interv. 2005; 65: 69-72Crossref PubMed Scopus (64) Google Scholar In all reports published to date, a percutaneous approach was used. In our case, such an approach was not possible because the patient had two mechanical valves. Passing a mechanical aortic prosthesis with a big, stiff sheath leads to significant aortic insufficiency. Furthermore, no valve company guarantees that the leaflets of the valves will stay in place when passing them with a stiff sheath. Another option, the antegrade approach to the mitral valve through a puncture in the atrial septum, is also extremely difficult, because the antegrade pathway to the mitral valve is very curvy. In our case we discussed the options: a standard reoperation with median sternotomy, right lateral thoracotomy with cardiopulmonary bypass, or an off-pump hybrid procedure. As there was no need to exchange the well-functioning mitral prosthesis itself, we decided to go for the last option. Approaching the paravalvular leak from the left ventricular apex turned out to be much easier than expected. The guidewire, which was approached first, fell more or less by itself into the dehiscence between the sewing ring and annulus. However, there was one delicate situation. During the pushing of the Amplatzer device out of the sheath, one leaflet of the mitral valve was blocked. It was hard to determine whether the umbrella itself or the sheath was responsible was responsible for this blockade. We pulled the introducer sheath back without detaching the umbrella. Fortunately, valve function returned to normal, proving that the sheath did indeed compromise function. We believe that in this particular situation, a hybrid procedure, reduced the risk of the operative procedure significantly.

We report on a rare case of combined unilateral atresia of the proximal right pulmonary artery (PA) and left patent ductus arteriosus (PDA). A 46 year-old female patient with known PDA and associated advanced pulmonary arterial hypertension presented with progressive hemoptysis and increasing exertional dyspnea. Computed tomography of the chest proved the presence of the known PDA but surprisingly failed to demonstrate an extrapulmonary proximal right PA. We show the imaging features and discuss the embryological background of this rare congenital cardiovascular malformation.

The high incidence of sudden cardiac death in heart failure (HF) reflects electrophysiologic changes in response to myocardial failure. We previously showed that short-term variability of QT intervals (STV(QT)) identifies latent repolarization disorders in patients with drug-induced or congenital long QT syndrome. This study sought to determine (1) if STV(QT) is increased in patients with dilated cardiomyopathy (DC) and moderate congestive HF and (2) if increased STV(QT) is associated with ventricular arrhythmia in patients with HF. Sixty patients (53 +/- 12 years of age, 14 women) with DC and moderate HF (New York Heart Association classes II to III) were compared to matched controls. Twenty patients had implantable cardiac defibrillators secondary to a history of ventricular tachycardia (VT). Two cardiologists blinded to diagnosis manually measured QT intervals. Beat-to-beat variability of repolarization was determined from Poincaré plots of 30 consecutive QT intervals as was STV(QT). QTc intervals were comparable in patients and controls (419 +/- 36 vs 415 +/- 32 ms, respectively, p >0.05), whereas STV(QT) was significantly higher in patients with HF (7.8 +/- 3 vs 4.1 +/- 2 ms, respectively, p <0.05). STV(QT) was more increased in patients with a history of VT compared to those without VT (10.1 +/- 2 vs 6.6 +/- 2 ms, respectively, p <0.05). Increased STV(QT) and decreased ejection fraction were associated with a history of VT; however, STV(QT) was the strongest indicator. In conclusion, the present study demonstrates for the first time that STV(QT) is increased in patients with DC with HF. Patients with DC and HF and implantable cardiac defibrillators for secondary prevention had the highest STV(QT). Thus, increased STV(QT) in the context of moderate HF may reflect a latent repolarization disorder and increased susceptibility to sudden death in patients with DC, which is not identified by a prolonged QT interval.

Vorgestellt wird der Fall eines 27-jährigen Mannes, der im Rahmen eines fieberhaften Infekts synkopiert ist. Ein vom Notarzt geschriebenes 12-Kanal-EKG zeigte – typisch für ein Brugada-Syndrom – einen erhöhten ST-Abgang in den Ableitungen V1–V3. Unter fiebersenkender Therapie bildeten sich die EKG-Veränderungen zurück. Die Diagnose Brugada-Syndrom konnte durch einen Ajmalintest bestätigt werden. Hierbei handelt es sich um eine seltene arrhythmogene Erkrankung, die in 30% der Fälle mit einer Mutation im SCN5A-Gen assoziiert ist, welches für den myokardialen Natriumkanal kodiert. Dem Patienten wurde nach auffälliger elektrophysiologischer Untersuchung ein implantierbarer Kardioverter/Defibrillator (ICD) implantiert.

46-year-old female patient presented at the emergencydepartment with hemoptysis of unknown cause. Pastmedical history was significant for pulmonary hypertensionwith Eisenmenger syndrome due to a patent ductus arteriosusthat had been diagnosed in childhood. Additionally, mildasthma was reported. Except for mild dyspnea and coughing,the patient was in good general condition and did not showany signs of respiratory infection. Abdominal hysterectomyhad been performed due to a uterine myoma 1 year before.Physical examination showed differential central cyanosiswith clubbing of the toes but not of the fingers. Cardiacauscultation revealed an accentuated second heart sound atthe base and a 2/6 continuous machinery murmur at the upperleft sternal region. On lung auscultation, left-sided basilarrales were noted, but no wheezing. Pertinent laboratoryresults were unremarkable except for microcytic, hypochro-mic polycythemia (red blood cells: 7.96 610 / L, meancorpuscular volume 74.7 fL, mean corpuscular hemoglobin23.6 pg/cell). Blood oxygen saturation in the upper extremi-ties was 96%.The patient was referred for CT angiography of thepulmonary arteries to locate the origin of hemoptysis. A CTscan was performed on a dual-source CT system (SomatomDefinition, Siemens Medical Systems, Forchheim, Germany)in dual-energy mode at 140 and 80 kVp tube potential afterinjection of 80 mL of high-concentration iodinated contrastmaterial (Iopromide, Ultravist 370, Bayer Schering PharmaAG, Berlin, Germany). This recently developed CT techniquemakes it possible to detect iodine by its spectral properties,thus enabling direct visualization of the perfusion of lungparenchyma.

Apart from clinical symptoms the diagnosis and risk stratification in long-QT syndrome (LQTS) is usually based on the surface electrocardiogram. Studies have indicated that not only prolongation of the QT interval but also an increased short-term variability of QT interval (STV(QT)) is a marker for a decreased repolarization reserve in patients with drug-induced LQTS. The aims of this study were to determine if STV(QT) (1) is higher in patients with LQTS compared with controls, (2) if this effect is more pronounced in a high-risk LQTS population, and (3) could increase the diagnostic power of the surface electrocardiogram in identifying mutation carriers. Forty mutation carriers were compared with age- and gender-matched control subjects in the absence of beta-receptor-blocking agents. Lead II or V(5) RR and QT intervals from 30 consecutive beats were manually measured. STV(QT) was determined from Poincaré plots of QT intervals (STV(QT) = Sigma|QTn + 1 - QTn|/[30 x radical2]). Compared with controls, patients with LQTS had a prolonged QTc interval (449 +/- 41 vs 411 +/- 32 ms, p = 0.00049) and increased STV(QT) (6.4 +/- 3.2 vs 4.1 +/- 1.6 ms, p = 0.005). In patients with the highest risk of clinical events, defined as a QTc interval >500 ms or symptoms before beta-blocker therapy, STV(QT) was 9 +/- 4 ms. QTc interval had a sensitivity of 43% and a specificity of 97% in identifying mutation carriers (thresholds 450 ms for men and 460 ms for women). Receiver operator characteristic analysis showed that an STV(QT) of 4.9 ms was the optimal cut-off value to predict mutation carriers. When incorporating an STV(QT) >4.9 ms for those whose QTc interval was within the normal limits, sensitivity to distinguish mutation carriers increased to 83% with a specificity of 68%, so that another 15 mutation carriers could be identified. In conclusion, these are the first results in humans showing that STV(QT) is increased in congenital LQTS, this effect is increased in patients with symptoms before therapy, and, hence, STV(QT) could prove to be a useful noninvasive additive marker for diagnostic screening to bridge the gap before results of genetic testing are available.

The authors investigated whether computerized parameters quantifying ventricular repolarization delay, heterogeneity, and instability characterize individuals who developed drug‐induced Torsades de Pointes. Assessing an individual's propensity to Torsades de Pointes when exposed to a QT‐prolonging drug is challenging because baseline QT prolongation has limited predictive value. Five‐minute digital 12‐lead electrocardiograms were acquired at baseline and after a sotalol challenge in 16 patients who had a history of Torsades de Pointes in the context of a QT‐prolonging drug and 17 patients who did not have such history. Computerized measurements of QTc, T peak to T end intervals (TpTe), TpTe/QTc, and QT variability were implemented, and novel quantifiers of ventricular repolarization heterogeneity from the early (ERD) and late (LRD) part of the T wave were investigated. Compared with electrocardiograms of patients without a history of Torsades de Pointes, the baseline electrocardiograms of patients with a history of Torsades de Pointes had a longer QTc and an increased repolarization heterogeneity of the early part of the T wave (ERD 30% : 44 ± 13 vs 35 ± 8 ms, P = .02). On sotalol, the electrocardiograms from individuals with Torsades de Pointes revealed a delay of the terminal part of the T wave that was not present in patients without Torsades de Pointes (TpTe: 27 ± 40 vs −1 ± 21 ms, P = .02; LRD 70% : 20 ± 29 vs 2 ± 4 ms, P = .04). Results suggest that the electrocardiogram abnormalities characterizing patients with a history of Torsades de Pointes are (1) an increased repolarization heterogeneity at baseline and (2) a sotalol‐induced prolongation of the terminal part of the T wave.

Background: The long‐QT syndromes (LQTS) are inherited electrical cardiomyopathies characterized by prolonged ventricular repolarization and ventricular arrhythmias. Several genetic reports have associated defects in LQTS‐causing genes with atrial fibrillation (AF). We therefore studied whether atrial arrhythmias occur in patients with LQTS under daily‐life conditions. Methods: We systematically assessed atrial arrhythmias in LQTS patients and matched controls using implanted defibrillators or pacemakers as monitors of atrial rhythm in a nested case‐control study. Twenty‐one LQTS patients (3 male; 39 ± 18 years old; 18 on β blocker, ICD therapy duration 6.3 ± 2.7 years; 4 LQT1, 6 LQT2, 2 LQT3) were matched to 21 control subjects (13 male; 50 ± 19 years old; 3 on β blocker; pacemaker therapy duration 8.5 ± 5.5 years; 19 higher‐degree AV block, 2 others). LQTS patients were identified by a systematic search of the LQTS patient databases in Münster and Munich. Results: One‐third (7 of 21) of the LQTS patients developed self‐terminating atrial arrhythmias (atrial cycle lengths <250 ms). Only one control patient developed a single episode of postoperative AF (P < 0.05 vs LQTS). Conclusions: LQTS patients at high risk for ventricular arrhythmias may develop short‐lasting atrial arrhythmias under daily‐life conditions, suggesting that prolonged atrial repolarization may contribute to the initiation of AF.

HomeCirculationVol. 119, No. 8Unilateral Pulmonary Artery Agenesis Free AccessReview ArticlePDF/EPUBAboutView PDFView EPUBSections ToolsAdd to favoritesDownload citationsTrack citationsPermissionsDownload Articles + Supplements ShareShare onFacebookTwitterLinked InMendeleyReddit Jump toSupplementary MaterialsFree AccessReview ArticlePDF/EPUBUnilateral Pulmonary Artery AgenesisNoninvasive Diagnosis With Dual-Source Computed Tomography T.R.C. Johnson, MD, S.F. Thieme, MD, M. -A. Deutsch, MD, M. Hinterseer, MD, M.F. Reiser, MD, C.R. Becker, MD and K. Nikolaou, MD T.R.C. JohnsonT.R.C. Johnson From the Institut für Klinische Radiologie der LMU München, Klinikum Großhadern (T.R.C.J., S.F.T., M.F.R., C.R.B., K.N.), and Medizinische Klinik und Poliklinik I Großhadern der LMU München (M.-A.D., M.H.), Munich, Germany. , S.F. ThiemeS.F. Thieme From the Institut für Klinische Radiologie der LMU München, Klinikum Großhadern (T.R.C.J., S.F.T., M.F.R., C.R.B., K.N.), and Medizinische Klinik und Poliklinik I Großhadern der LMU München (M.-A.D., M.H.), Munich, Germany. , M. -A. DeutschM. -A. Deutsch From the Institut für Klinische Radiologie der LMU München, Klinikum Großhadern (T.R.C.J., S.F.T., M.F.R., C.R.B., K.N.), and Medizinische Klinik und Poliklinik I Großhadern der LMU München (M.-A.D., M.H.), Munich, Germany. , M. HinterseerM. Hinterseer From the Institut für Klinische Radiologie der LMU München, Klinikum Großhadern (T.R.C.J., S.F.T., M.F.R., C.R.B., K.N.), and Medizinische Klinik und Poliklinik I Großhadern der LMU München (M.-A.D., M.H.), Munich, Germany. , M.F. ReiserM.F. Reiser From the Institut für Klinische Radiologie der LMU München, Klinikum Großhadern (T.R.C.J., S.F.T., M.F.R., C.R.B., K.N.), and Medizinische Klinik und Poliklinik I Großhadern der LMU München (M.-A.D., M.H.), Munich, Germany. , C.R. BeckerC.R. Becker From the Institut für Klinische Radiologie der LMU München, Klinikum Großhadern (T.R.C.J., S.F.T., M.F.R., C.R.B., K.N.), and Medizinische Klinik und Poliklinik I Großhadern der LMU München (M.-A.D., M.H.), Munich, Germany. and K. NikolaouK. Nikolaou From the Institut für Klinische Radiologie der LMU München, Klinikum Großhadern (T.R.C.J., S.F.T., M.F.R., C.R.B., K.N.), and Medizinische Klinik und Poliklinik I Großhadern der LMU München (M.-A.D., M.H.), Munich, Germany. Originally published3 Mar 2009https://doi.org/10.1161/CIRCULATIONAHA.108.777698Circulation. 2009;119:1158–1160A 46-year-old female patient presented at the emergency department with hemoptysis of unknown cause. Past medical history was significant for pulmonary hypertension with Eisenmenger syndrome due to a patent ductus arteriosus that had been diagnosed in childhood. Additionally, mild asthma was reported. Except for mild dyspnea and coughing, the patient was in good general condition and did not show any signs of respiratory infection. Abdominal hysterectomy had been performed due to a uterine myoma 1 year before. Physical examination showed differential central cyanosis with clubbing of the toes but not of the fingers. Cardiac auscultation revealed an accentuated second heart sound at the base and a 2/6 continuous machinery murmur at the upper left sternal region. On lung auscultation, left-sided basilar rales were noted, but no wheezing. Pertinent laboratory results were unremarkable except for microcytic, hypochromic polycythemia (red blood cells: 7.96×106/μL, mean corpuscular volume 74.7 fL, mean corpuscular hemoglobin 23.6 pg/cell). Blood oxygen saturation in the upper extremities was 96%.The patient was referred for CT angiography of the pulmonary arteries to locate the origin of hemoptysis. A CT scan was performed on a dual-source CT system (Somatom Definition, Siemens Medical Systems, Forchheim, Germany) in dual-energy mode at 140 and 80 kVp tube potential after injection of 80 mL of high-concentration iodinated contrast material (Iopromide, Ultravist 370, Bayer Schering Pharma AG, Berlin, Germany). This recently developed CT technique makes it possible to detect iodine by its spectral properties, thus enabling direct visualization of the perfusion of lung parenchyma.1CT angiography images showed the known patent ductus arteriosus but surprisingly revealed an absence of the right pulmonary artery. Blood supply to the right lung was only provided by compensatorily enlarged bronchial arteries (Figure 1), whereas the vascular morphology of the left lung, with tortuous, dilated central arteries, was typical for pulmonary arterial hypertension (Figure 2). The hypertrophy of the right ventricular myocardium was consistent with this diagnosis (Figure 3). There was a discrete edema of the left lung, presumably due to hyperperfusion (Figure 4) because of the unilateral perfusion, which was further increased by the patent ductus arteriosus (Figure 5). The spectral differentiation of iodine in the lung parenchyma on the basis of the dual-energy information showed an asymmetrical perfusion of the lungs with very limited enhancement of the right lung and normal perfusion on the left side (Figure 6). Download figureDownload PowerPointFigure 1. Volume-rendering technique image of the pulmonary CT angiography (right hemithorax). Note the absence of the right pulmonary artery and slightly dilated bronchial arteries.Download figureDownload PowerPointFigure 2. Volume-rendering technique image of the left hemithorax. Tortuous, dilated central pulmonary arteries indicate pulmonary hypertension.Download figureDownload PowerPointFigure 3. Axial CT image shows hypertrophy of the right ventricular myocardium (arrow), which indicates right ventricular strain.Download figureDownload PowerPointFigure 4. Axial image in lung window again shows dilated central pulmonary arteries on the left side. There is moderate blurring around the vessels, which indicates discrete edema.Download figureDownload PowerPointFigure 5. Sagittal reconstruction showing the descending aorta. The patent ductus arteriosus is confirmed, which implies a shunt between the descending aorta (*) and the enlarged left pulmonary artery (**).Download figureDownload PowerPointFigure 6. Visualization of the pulmonary perfusion based on detection of iodine by its spectral properties in dual-energy CT. The axial (A) and coronal (B) reconstruction reveal a hypoperfusion of the right lung, whereas the left lung is perfused normally.Unilateral agenesis of the pulmonary artery was first described in 1868,2 and some 90 cases have been reported since then.3 It represents a rare developmental anomaly due to a failure in the connection of the sixth aortic arch with the pulmonary trunk.4 Quite a few patients appear to remain asymptomatic until adulthood; however, an increased incidence of asthma and recurrent respiratory tract infections has been noted. Some patients also present with dyspnea on exertion or congestive heart failure.5,6 Hemoptysis has been described as a clinical symptom in up to 10% of patients, originating from either hypertrophied bronchial collateral vessels or peripheral arteriovenous fistulas ipsilateral to the absent pulmonary artery, as well as from rupture of chronically hyperperfused vessels on the contralateral side.7 There have been reports of a successful surgical reconstruction of a pulmonary artery in early childhood. A therapeutic option in adults may be an interventional or surgical occlusion of collateral vessels or pneumonectomy on the affected side to avoid hemoptysis. Heart-lung transplantation may be an option to simultaneously improve blood oxygenation.8 In our patient, the combination of continued hemoptysis, pulmonary hypertension, and exertion on exercise indicated that heart-lung transplantation was the most suitable therapeutic option.*The first 2 authors contributed equally to this work.The online-only Data Supplement is available with this article at http://circ.ahajournals.org/cgi/content/full/119/8/1158/DC1.DisclosuresNone.FootnotesCorrespondence to Dr Sven F. Thieme, MD, Institut für Klinische Radiologie der LMU München, Klinikum Großhadern, Marchioninistraße 15, D-81377 München, Germany. E-mail [email protected]References1 Johnson TR, Krauss B, Sedlmair M, Grasruck M, Bruder H, Morhard D, Fink C, Weckbach S, Lenhard M, Schmidt B, Flohr T, Reiser MF, Becker CR. Material differentiation by dual energy CT: initial experience. Eur Radiol. 2007; 17: 1510–1517.CrossrefMedlineGoogle Scholar2 Fraentzel O. Angeborener defect der rechten lungenarterie. Virchows Arch Pathol Anat. 1868; 43: 420.CrossrefGoogle Scholar3 Ohta T. Congenital absence of the right pulmonary artery: a case report and review. Tokai J Exp Clin Med. 1983; 8: 97–104.MedlineGoogle Scholar4 Cucci CE, Doyle EF, Lewis EW Jr. Absence of a primary division of the pulmonary trunk. an ontogenetic theory. Circulation. 1964; 29: 124–131.CrossrefMedlineGoogle Scholar5 Bouros D, Pare P, Panagou P, Tsintiris K, Siafakas N. The varied manifestation of pulmonary artery agenesis in adulthood. Chest. 1995; 108: 670–676.CrossrefMedlineGoogle Scholar6 Ten Harkel AD, Blom NA, Ottenkamp J. Isolated unilateral absence of a pulmonary artery: a case report and review of the literature. Chest. 2002; 122: 1471–1477.CrossrefMedlineGoogle Scholar7 Lip GY, Dunn FG. Unilateral pulmonary artery agenesis: a rare cause of haemoptysis and pleuritic chest pain. Int J Cardiol. 1993; 40: 121–125.CrossrefMedlineGoogle Scholar8 Trivedi KR, Freedom RM, Yoo SJ. Distal ductal origin of the pulmonary artery. In: Freedom RM, Yoo S-J, Mikailian H, Williams WG. The Natural and Modified History of Congenital Heart Disease. Chapter 8. Elmsford, NY: Blackwell; 2004.Google Scholar Previous Back to top Next FiguresReferencesRelatedDetailsCited By Jariwala P, Maturu V, Christopher J and Jadhav K (2020) Congenital isolated unilateral agenesis of pulmonary arteries in adults: case series and review, Indian Journal of Thoracic and Cardiovascular Surgery, 10.1007/s12055-020-01032-w, 37:S1, (144-154), Online publication date: 1-Jan-2021. Choi Y, Kang N, Im Y, Choe J, Kim T and Kim H (2019) Unilateral pulmonary artery agenesis manifested by hemoptysis in pregnancy, Respiratory Medicine Case Reports, 10.1016/j.rmcr.2018.10.028, 26, (14-16), . Seedat F, Kalla I and Feldman C (2017) Unilateral absent pulmonary artery in an adult - A diagnostic and therapeutic challenge, Respiratory Medicine Case Reports, 10.1016/j.rmcr.2017.09.004, 22, (238-242), . Meinel F, Zhang L and Schoepf U (2016) Dual-Energy CT of the Thorax Multidetector-Row CT of the Thorax, 10.1007/978-3-319-30355-0_15, (283-310), . Meinel F, Zhang L, Krazinski A and Schoepf U (2015) Dual-Energy CT in Thoracic Imaging Dual-Energy CT in Cardiovascular Imaging, 10.1007/978-3-319-21227-2_7, (95-112), . Almonacid Sánchez C (2014) Patología genética y congénita bronquial y pulmonar, Medicine - Programa de Formación Médica Continuada Acreditado, 10.1016/S0304-5412(14)70876-5, 11:67, (4012-4024), Online publication date: 1-Nov-2014. Betigeri V, Betigeri A, Saichandran B and SubbaRao K (2012) Bullous lung disease and bronchiectasis in unilateral absent right pulmonary artery, General Thoracic and Cardiovascular Surgery, 10.1007/s11748-012-0107-2, 61:2, (100-103), Online publication date: 1-Feb-2013. Lozano Rodríguez Á, Junquera Rionda P, Santana Montesdeoca J and Fornell Pérez R (2011) Solución del caso 33. Interrupción proximal unilateral de la arteria pulmonar izquierda, Radiología, 10.1016/j.rx.2010.12.002, 53:5, (476-479), Online publication date: 1-Sep-2011. March 3, 2009Vol 119, Issue 8 Advertisement Article InformationMetrics https://doi.org/10.1161/CIRCULATIONAHA.108.777698PMID: 19255355 Originally publishedMarch 3, 2009 PDF download Advertisement SubjectsComputerized Tomography (CT)Heart FailurePulmonary HypertensionTreatment