著者
Toru Miyoshi Kazuhiro Osawa Hiroshi Ito Susumu Kanazawa Takeshi Kimura Hiroki Shiomi Sachio Kuribayashi Masahiro Jinzaki Akio Kawamura Hiram Bezerra Stephan Achenbach Bjarne L. Nørgaard
出版者
日本循環器学会
雑誌
Circulation Journal (ISSN:13469843)
巻号頁・発行日
vol.79, no.2, pp.406-412, 2015-01-23 (Released:2015-01-23)
参考文献数
29
被引用文献数
5 23

Background:Recently, a non-invasive method using computational fluid dynamics to calculate vessel-specific fractional flow reserve (FFRCT) from routinely acquired coronary computed tomography angiography (CTA) was described. The Analysis of Coronary Blood Flow Using CT Angiography: Next Steps (NXT) trial, which was a prospective, multicenter trial including 254 patients with suspected coronary artery disease, noted high diagnostic performance of FFRCTcompared with invasive FFR. The aim of this post-hoc analysis was to assess the diagnostic performance of non-invasive FFRCTvs. standard stenosis quantification on coronary CTA in the Japanese subset of the NXT trial.Methods and Results:A total of 57 Japanese participants were included from Okayama University (n=36), Kyoto University (n=17), and Keio University (n=4) Hospitals. Per-patient diagnostic accuracy of FFRCT(74%; 95% confidence interval [CI]: 60–85%) was higher than for coronary CTA (47%; 95% CI: 34–61%, P<0.001) arising from improved specificity (63% vs. 27%, P<0.001). FFRCTcorrectly reclassified 53% of patients and 63% of vessels with coronary CTA false positives as true negatives. When patients with Agatston score >1,000 were excluded, per-patient accuracy of FFRCTwas 83% with a high specificity of 76%, similar to the overall NXT trial findings.Conclusions:FFRCThas high diagnostic performance compared with invasive FFR in the Japanese subset of patients in the NXT trial. (Circ J 2015; 79: 406–412)
著者
Lin Chang Hamblin Milton Daniel T. Eitzman Y. Eugene Chen
出版者
日本循環器学会
雑誌
Circulation Journal (ISSN:13469843)
巻号頁・発行日
vol.77, no.1, pp.11-18, 2013 (Released:2012-12-25)
参考文献数
74
被引用文献数
17 63

Perivascular adipose tissue (PVAT) is the fat tissue surrounding most of the vasculature and it has long been considered solely as vessel-supporting connective tissue. There are 2 major types of adipose tissue widely distributed throughout the body: white (WAT) and brown (BAT). PVAT is similar to BAT in rodents, but it was believed that only WAT existed in adult humans and BAT was present only in infants. However, the presence of functional BAT in adult humans is now accepted. The main function of BAT is to generate heat, and it is essential for adaptive thermogenesis and energy expenditure, whereas the main function of WAT is to store lipids. Besides the different functions of WAT and BAT, growing evidence suggests that different depots of adipose tissue have different functions. Similar to other fat depots, PVAT produces various adipokines, growth factors and inhibitors that affect functions of adjacent layers of the vasculature. Pathophysiological conditions such as obesity, vascular injury, aging and infection could cause PVAT dysfunction, leading to vascular endothelial and smooth muscle cell dysfunctions. In this review, we discuss the function and dysfunction of PVAT on atherosclerosis and hypertension.  (Circ J 2013; 77: 11–18)
著者
Takashi Kohno
出版者
日本循環器学会
雑誌
Circulation Journal (ISSN:13469843)
巻号頁・発行日
pp.CJ-14-1329, (Released:2014-12-15)
参考文献数
17
被引用文献数
12 16

The American Heart Association (AHA) Scientific Sessions were held in Chicago on November 15–19, 2014. The meeting attracted more than 17,000 participants, including physicians, research scientists, students, and paramedical personnel, from more than 100 countries. Sessions over the 5 days included comprehensive and unparalleled education delivered via more than 5,000 presentations, with 1,000 invited faculty members and 4,000 abstract presentations from world leaders in cardiovascular (CV) disease. There were 16 trials scheduled in 4 late-breaking clinical trial sessions. The Dual Antiplatelet Therapy study revealed that aspirin plus a thienopyridine beyond 1 year subsequent to placement of a drug-eluting stent, as compared with aspirin therapy alone, significantly reduced stent thrombosis and major CV and cerebrovascular events but was associated with increased risk of bleeding. The IMPROVE-IT research showed that, relative to simvastatin with placebo, simvastatin with 10 mg of ezetimibe daily led to a significantly lower primary combined endpoint in moderate- to high-risk patients, who stabilized following acute coronary syndrome. This was the first trial to demonstrate incremental clinical benefit by adding a nonstatin agent to statin therapy and reaffirmed the low-density lipoprotein (LDL) hypothesis stating that reducing LDL-cholesterol prevents CV events. Summaries and overviews of both the late-breaking trials and the sessions to which members of the Japanese Circulation Society contributed are presented.
著者
Hongzeng Xu Yuanzhe Jin Haifeng Ni Shengda Hu Qin Zhang
出版者
日本循環器学会
雑誌
Circulation Journal (ISSN:13469843)
巻号頁・発行日
vol.78, no.12, pp.2979-2986, 2014-11-25 (Released:2014-11-25)
参考文献数
43
被引用文献数
2 11

Background:Impairment of coronary flow reserve (CFR) has been generally demonstrated in diabetic patients and animals with microvascular complications but without obvious obstructive coronary atherosclerosis. There have been few studies investigating CFR in cases of relatively well-controlled therapy. The purpose of this study is to evaluate the effect of treatment with a Sphingosine-1-phosphate (S1P) receptor potent agonist, FTY720, on early diabetic rats in terms of CFR.Methods and Results:Male Sprague-Dawley (SD) rats were divided into 3 groups: (1) streptozotocin-uninjected rats (control rats); (2) streptozotocin-injected hyperglycemic rats (diabetic group); and (3) FTY720-fed and streptozotocin-injected hyperglycemic rats. FTY720 (1.25 mg/kg per day orally) was administrated for 9 weeks in SD rats (from 6 weeks old to 15 weeks old). CFR was evaluated by 13NH3-positron emission tomography. No obvious pathological changes of macrovascular atherosclerosis were observed in each group. Diabetic rats had impaired CFR compared with the control group (1.39±0.26 vs. 1.94±0.24, P<0.05). Treatment with FTY720 for 9 weeks attenuated the heart histological changes and improved CFR in 32% of diabetic rats (1.84±0.36 vs. 1.39±0.26, P<0.05).Conclusions:In summary, long-term therapy with the Sphingosine-1-phosphate receptor agonist, FTY720, improved CFR by attenuating the heart histological changes, and it might have a beneficial effect on coronary microvascular function in diabetic rats. (Circ J 2014; 78: 2979–2986)
著者
Akihiko Shimizu
出版者
日本循環器学会
雑誌
Circulation Journal (ISSN:13469843)
巻号頁・発行日
vol.78, no.12, pp.2851-2853, 2014-11-25 (Released:2014-11-25)
参考文献数
13
被引用文献数
3 3
著者
Tsugutoshi Suzuki Kyoichi Nishigaki
出版者
日本循環器学会
雑誌
Circulation Journal (ISSN:13469843)
巻号頁・発行日
vol.78, no.12, pp.2848-2850, 2014-11-25 (Released:2014-11-25)
参考文献数
11
被引用文献数
1 1
著者
Masashi Kanemoto Hiroko Kuhara Toru Ueda Takahiro Shinohara Takamasa Oda Fumiaki Nakao Toshiaki Kamei Yasuhiro Ikeda Takashi Fujii
出版者
日本循環器学会
雑誌
Circulation Journal (ISSN:13469843)
巻号頁・発行日
vol.78, no.11, pp.2651-2656, 2014-10-24 (Released:2014-10-24)
参考文献数
20
被引用文献数
3 13

Background:This study evaluated whether measuring prothrombin time (PT) using particular reagents of interest predicted apixaban-associated anticoagulant activity in Japanese patients with non-valvular atrial fibrillation (NVAF).Methods and Results:Two reagents, Shinplastin Excel S and Coagpia PT-N, were used to evaluate PT under apixaban therapy. From June 2013 to February 2014, 103 NVAF patients were recruited, and PT was measured at 3 time points: (1) anytime in the outpatient clinic, (2) at peak, and (3) at trough. In spike-in experiments using pooled citrated normal human platelet-poor plasma with these PT reagents, apixaban prolonged PT values in a concentration-dependent manner. PT values significantly correlated between both reagents (r=0.97) in outpatients. PT values in outpatients taking 5-mg apixaban bid were significantly prolonged and had wide inter- and intraindividual variability. Peak values were significantly higher than trough values, with both values higher than normal. The dose change of apixaban from 5 mg bid to 2.5 mg bid in outpatients halved the degree of PT prolongation in each NVAF patient.Conclusions:The PT value measured by these specific reagents can predict apixaban-associated anticoagulant activity, although there is significant interpatient variability. (Circ J 2014; 78: 2651–2656)
著者
Takuo Emoto Naoto Sasaki Tomoya Yamashita Kazuyuki Kasahara Keiko Yodoi Yoshihiro Sasaki Takuya Matsumoto Taiji Mizoguchi Ken-ichi Hirata
出版者
日本循環器学会
雑誌
Circulation Journal (ISSN:13469843)
巻号頁・発行日
pp.CJ-14-0644, (Released:2014-10-18)
参考文献数
22
被引用文献数
9 40

Background:The protective function of regulatory T cells (Treg) has been identified in experimental atherosclerosis, but the contribution of Tregto the pathogenesis of human coronary artery disease (CAD) remains poorly understood. We investigated Tregand regulatory T-cell/effector T-cell (Treg/Teff) ratio in peripheral blood samples from CAD patients using a new strategy for precise identification of Treg.Methods and Results:Peripheral blood samples were collected from 73 stable CAD patients (55 middle-aged CAD patients and 18 old CAD patients) and 64 controls (47 middle-aged controls and 17 young controls). CD3+CD4+FoxP3+T cells were divided into 3 fractions: CD45RA+FoxP3lowresting Treg(Fr1), CD45RA–FoxP3highactivated Treg(Fr2), and CD45RA–FoxP3lownon-Treg(Fr3). CAD patients had lower percentages of Fr1 and Fr2 and higher percentages of Fr3 and CD45RA–Foxp3–Teff(Fr4+5) within the CD3+CD4+T-cell population compared to age-matched controls. Treg/Teffratio (Fr1+2/Fr3+4+5) in CAD patients was also markedly lower than in controls (middle-aged control, 0.17±0.09 vs. middle-aged CAD, 0.10±0.05; P<0.001). The percentage of CD4+CD28nullT cells within the CD4+T-cell population was negatively correlated with Treg/Teffratio, excluding CD4+CD28nullT cells <0.3% (r=–0.27, P<0.05). High-sensitivity C-reactive protein was also negatively correlated with Treg/Teffratio (r=–0.22, P<0.05).Conclusions:CAD patients had reduced Tregand Treg/Teffratio compared to healthy controls. The present findings may be helpful when developing immunotherapy for the prevention of CAD.
著者
Yoshihisa Matsumura Jun Takata Hiroaki Kitaoka Toru Kubo Yuichi Baba Eri Hoshikawa Tomoyuki Hamada Makoto Okawa Nobuhiko Hitomi Kyoko Sato Naohito Yamasaki Toshikazu Yabe Takashi Furuno Masanori Nishinaga Yoshinori Doi
出版者
日本循環器学会
雑誌
Circulation Journal (ISSN:13469843)
巻号頁・発行日
vol.70, no.4, pp.376-383, 2006 (Released:2006-03-25)
参考文献数
42
被引用文献数
21 42

Background Because of their favorable prognostic effects, angiotensin converting enzyme inhibitors (ACEI), angiotensin II receptor blockers (ARB) and β blockers have become background therapy in dilated cardiomyopathy (DCM). However, there are few reports concerning the long-term prognosis of Japanese patients with DCM in relation to these treatments. Methods and Results One hundred and fifty patients with DCM were divided into 2 groups: group A (n=46) (diagnosis: 1982-1989) and group B (n=104) (diagnosis: 1990-2002). During follow-up period of 6.9±4.8 years, 62 patients died and 1 patient had a heart transplant. The survival rate at 5 and 10 years was 60.9% and 34.8%, respectively, in group A patients, and 80.9% and 65.3%, respectively, in group B patients (p=0.0079). In group A patients, ACEI/ARB or β blockers were less frequently used (p<0.0001), whereas antiarrhythmics (class Ia or Ib) were more often used (p<0.0001). The patients treated with ACEI/ARB and β blockers showed a better survival rate than those without (p<0.0001). The patients with antiarrhythmics showed a worse survival rate than those without (p<0.0001). Conclusion The prognosis of Japanese patients with DCM has significantly improved over the past 20 years. This improvement may be explained partly through the increased use of ACEI/ARB and β blockers and a declining use of antiarrhythmics. (Circ J 2006; 70: 376 - 383)
著者
Wataru Shimizu
出版者
日本循環器学会
雑誌
Circulation Journal (ISSN:13469843)
巻号頁・発行日
vol.77, no.12, pp.2867-2872, 2013 (Released:2013-11-25)
参考文献数
46
被引用文献数
15 41 4

Over the past 2 decades, a number of inherited cardiac arrhythmias, including congenital long QT syndrome (LQTS) and Brugada syndrome (BrS), have been shown to have a link to mutations in genes encoding for ion channels or other membrane components. The recent HRS/EHRA/APHRS expert consensus statement on the diagnosis and management of patients with inherited arrhythmia syndromes has updated the clinical diagnosis of congenital LQTS and BrS. Genetic studies have identified 13 forms of congenital LQTS in 50–80% of clinically affected patients. Genotype-phenotype correlations have been investigated in the 3 major genotypes, LQT1, LQT2 and LQT3 syndromes, resulting in genotype-specific management and therapy. More detailed analyses of each genotype have suggested mutation location-, type-, or function-specific differences in clinical phenotype among the LQT1, LQT2, and possibly LQT3 genotypes. In BrS, only one-third of affected patients can be genotyped, mainly in the sodium channel gene, SCN5A; therefore, clinical studies of genotype-phenotype relationships have been limited. More recently, a genome-wide association study using a gene array explored the role of common genetic variants (polymorphisms) as the susceptible or modifier gene in both congenital LQTS and BrS.  (Circ J 2013; 77: 2867–2872)
著者
Masaharu Akao Yeong-Hwa Chun Masahiro Esato Mitsuru Abe Hikari Tsuji Hiromichi Wada Koji Hasegawa on behalf of the Fushimi AF Registry Investigators
出版者
日本循環器学会
雑誌
Circulation Journal (ISSN:13469843)
巻号頁・発行日
pp.CJ-14-0344, (Released:2014-06-27)
参考文献数
20
被引用文献数
34 116

Background:Atrial fibrillation (AF) is a common arrhythmic disorder among the elderly, and increases the risk of stroke. Oral anticoagulants (OAC) are highly effective in preventing stroke, and there are evidence-based guidelines for the optimal use of OAC in patients with AF.Methods and Results:The Fushimi AF Registry is a community-based prospective survey of the AF patients in Fushimi-ku, Kyoto, a typical urban community in Japan with a total population of 283,000. Of the 3,282 patients enrolled by October 2012, 1-year follow-up was completed for 2,914 patients. OAC, mainly warfarin, were given to 1,546 patients (53.1%); overused for low-risk patients, and underused for patients at risk, based on the guidelines. Moreover, warfarin was sometimes given at a sub-therapeutic dose; only 54.4% of patients were within the optimal therapeutic range. The 1-year outcomes revealed that the incidences of both stroke and major bleeding were equivalent between patients taking OAC and those without; major clinical events were as follows: (OAC vs. non-OAC) stroke 2.7% vs. 2.8%, ischemic stroke 2.1% vs. 2.0% and major bleeding 1.4% vs. 1.5% (NS for all).Conclusions:The Fushimi AF Registry provides a unique snapshot of current AF management in an urban community in Japan. The present study reveals inappropriate use of OAC for patients with AF, indicating discordance between guideline recommendations and real-world clinical practice.
著者
Carlos E. Salinas Carlos E. Blanco Mercedes Villena Dino A. Giussani
出版者
日本循環器学会
雑誌
Circulation Journal (ISSN:13469843)
巻号頁・発行日
pp.CJ-13-1329, (Released:2014-04-16)
参考文献数
51
被引用文献数
2 18

Background: By combining the chick embryo model with incubation at high altitude (HA), the effects of chronic hypoxia on fetal growth, fetal cardiac and aortic wall remodeling and systemic arterial blood pressure at adulthood were reported. Using non-invasive functional echocardiography, here we investigated the in vivo effects of HA hypoxia on the pulmonary circulation at adulthood in male and female chickens. Methods and Results: Chick embryos were incubated, hatched and raised at sea level (SL) or at HA. At 6 months of age, functional echocardiography was performed and the body and heart weights were taken. Heart weight was heavier in males but not in female HA chickens compared to their same sex SL counterparts. Similarly, male but not female HA chickens had greater in vivo right ventricular wall thickness compared to their same sex SL counterparts. The tricuspid pressure gradient was greatly enhanced in HA male and HA female chickens. However, the increment in the tricuspid pressure gradient was greater in HA males than in HA females. The pulmonary artery diameter was also enhanced in HA males than in SL males. In contrast, HA did not affect this variable in female chickens. Conclusions: The data show that chronic hypoxia during development at HA is associated with echocardiocraphic indices of pulmonary hypertension at adulthood in a highly sex-dependent manner.
著者
Kaori Nakanishi Makoto Nishida Tohru Ohama Toshiki Moriyama Keiko Yamauchi-Takihara
出版者
日本循環器学会
雑誌
Circulation Journal (ISSN:13469843)
巻号頁・発行日
pp.CJ-13-1134, (Released:2014-03-11)
参考文献数
33
被引用文献数
5 21

Background: Smoking and metabolic syndrome (MetS) are major public health problems in modern society and are important risk factors of cardiovascular disease (CVD). The association of smoking, MetS, and CVD is widely reported, but reports targeted to women are few. In the present study, we evaluated risk factors, including visceral fat area (VFA), for CVD and development of subclinical atherosclerosis in female smokers especially. Methods and Results: Subjects consisted of 162 apparent healthy female and male smokers, and 315 age-matched never-smokers who underwent a health examination in the Osaka University Health Care Center. For female smokers, lifestyle and carotid intima-media thickness (IMT) were evaluated. Triglycerides were significantly higher and high-density lipoprotein-cholesterol significantly lower in smokers than in never-smokers for both men and women. However, VFA was significantly high only in smoking women when compared with never-smokers. Multivariate analysis revealed that age, body mass index, and smoking were the independent predictors of high VFA in women. In addition, annual IMT increase was significantly higher in smokers than never-smokers in women. Conclusions: VFA was notably high in female smokers, but the difference was not observed in men. Smoking habit is an important risk factor of visceral fat accumulation and progression of subclinical atherosclerosis in women.
著者
Yoko Miyasaka
出版者
日本循環器学会
雑誌
Circulation Journal (ISSN:13469843)
巻号頁・発行日
vol.77, no.7, pp.1681-1683, 2013 (Released:2013-06-25)
参考文献数
15
被引用文献数
2 2
著者
Ángel López-Cuenca Sergio Manzano-Fernández Francisco Marín Soledad Parra-Pallares Marina Navarro-Peñalver Salvador Montalban-Larrea Jose M. Andreu-Cayuelas Ana I. Romero-Aniorte Francisco Avilés-Plaza Mariano Valdés-Chavarri James L. Januzzi Jr
出版者
日本循環器学会
雑誌
Circulation Journal (ISSN:13469843)
巻号頁・発行日
vol.77, no.8, pp.2088-2096, 2013 (Released:2013-07-25)
参考文献数
24
被引用文献数
4 5

Background: Beta-trace protein (BTP) and cystatin C (CysC) are novel biomarkers of renal function. We assessed the ability of both to predict major bleeding (MB) in patients with non-ST-segment elevation acute coronary syndromes (NSTE-ACS), compared to other renal function parameters and clinical risk scores. Methods and Results: We included 273 patients. Blood samples were obtained within 24h of admission. The endpoint was MB. During a follow-up of 760 days (411–1,098 days), 25 patients (9.2%) had MB. Patients with MB had higher concentrations of BTP (0.98mg/L; 0.71–1.16mg/L vs. 0.72mg/L, 0.60–0.91mg/L, P=0.002), CysC (1.05mg/L; 0.91–1.30mg/L vs. 0.90mg/L, 0.75–1.08mg/L, P=0.003), higher CRUSADE score (39±16 points vs. 29±15 points, P=0.002) and lower estimated glomerular filtration rate (eGFR; 66±27 vs. 80±30ml·min–1·1.73m–2, P=0.02) than patients without MB; there was no difference in creatinine level between the groups (P=0.14). After multivariable adjustment, both were predictors of MB, while eGFR and creatinine did not achieve statistical significance. Among subjects with eGFR >60ml·min–1·1.73m–2, those with elevated concentrations of both biomarkers had a significantly higher risk for MB. Net reclassification indexes from the addition of BTP and CysC to CRUSADE risk score were 38% and 21% respectively, while the relative integrated discrimination indexes were 12.5% and 3.8%. Conclusions: Among NSTE-ACS patients, BTP and CysC were superior to conventional renal parameters for predicting MB, and improved clinical stratification for hemorrhagic risk.  (Circ J 2013; 77: 2088–2096)
著者
Miguel Ruiz-Canela Miguel A. Martínez-González
出版者
日本循環器学会
雑誌
Circulation Journal (ISSN:13469843)
巻号頁・発行日
pp.CJ-14-0062, (Released:2014-02-01)
参考文献数
101
被引用文献数
9 36

Peripheral artery disease (PAD) usually refers to ischemia of the lower limb vessels. Currently, the estimated number of cases in the world is 202 million. PAD is the third leading cause of atherosclerotic cardiovascular morbidity. The measurement of the ankle-brachial index (ABI) is recommended as a first-line noninvasive test for screening and diagnosis of PAD. An ABI <0.90 is an independent predictor of cardiovascular events and this measurement is useful to identify patients at moderate to high risk of cardiovascular disease. However, there is insufficient evidence to assess the benefits and harms of screening for PAD with the ABI in asymptomatic adults. Lifestyle modifications, including smoking cessation, dietary changes and physical activity, are currently the most cost-effective interventions. Inverse associations with PAD have been reported for some subtypes of dietary fats, fiber, antioxidants (vitamins E and C), folate, vitamins B6, B12 and D, flavonoids, and fruits and vegetables. A possible inverse association between better adherence to the Mediterranean diet and the risk of symptomatic PAD has also been reported in a large randomized clinical trial. Therefore, a Mediterranean-style diet could be effective in the primary and secondary prevention of PAD, although further experimental studies are needed to better clarify this association.
著者
Yae Matsuo Marcus Sandri Norman Mangner Nicolas Majunke Ingo Dähnert Gerhard Schuler Masahiko Kurabayashi Sven Möbius-Winkler
出版者
日本循環器学会
雑誌
Circulation Journal (ISSN:13469843)
巻号頁・発行日
pp.CJ-13-0828, (Released:2014-01-11)
参考文献数
29
被引用文献数
7 17

Background: Transcatheter left atrial appendage (LAA) closure is an alternative therapy for stroke prevention in atrial fibrillation (AF) patients. However; real-world efficacy, safety and complications have yet to be investigated. We sought to determine the procedural outcomes and potential complications of LAA closure in routine clinical practice at a high-volume center. Methods and Results: The study group comprised 179 patients (105 males; 72.7±9.0 years) with AF undergoing LAA closure at a single center in Germany. The rate of successful implantation was 98.9% (2 patients did not undergo implantation) and the overall procedure-related complication rate was 11.2% (major: 3.3%: tamponade 2; possibility of transient ischemic attack (TIA) 1; device dislocation 3; minor: 7.8%: pericardial effusion 2; air embolization with transient ST segment elevation 3; thrombus on device/sheath 3; puncture complications 5). At 45 days; 99.4% showed successful sealing of the LAA and 94.5% discontinued oral anticoagulation (OAC). TIA occurred in 2 patients during 6-month follow-up; but no cases of stroke were reported. There were no hemorrhagic stroke or device-related deaths. Only 1 patient was hospitalized with traumatic subdural hematoma. Minor bleeding was reported in 5 patients. Conclusions: Transcatheter LAA closure in a high-volume center is safe and feasible. Life-threatening complications are rare. Discontinuation of OAC 45 days after implantation was also safe.
著者
Patrick O. Myers Cécile Tissot Maurice Beghetti
出版者
日本循環器学会
雑誌
Circulation Journal (ISSN:13469843)
巻号頁・発行日
vol.78, no.1, pp.4-11, 2014 (Released:2013-12-25)
参考文献数
96
被引用文献数
7 42

Pulmonary arterial hypertension (PAH) is a common complication of congenital heart disease, and is now predominantly among patients with uncorrected left-to-right shunts. A growing population is characterized by persistent or recurrent PAH after surgical or interventional correction of left-to-right shunts; the latter having a worse prognosis than other forms of PAH associated with congenital heart disease. New treatments for PAH have been shown to be effective in improving PAH exercise capacity and hemodynamics, raising the hope for making previously inoperable congenital heart defects operable and shifting the framework for the assessment of operability. This review focuses on current methods for assessing operability in PAH associated with congenital heart disease, and the possibility of “treat-and-repair” vs. “repair-and-treat” strategies for patients with inoperable or borderline PAH.  (Circ J 2014; 78: 4–11)