Tomoaki Murakami

Aortic surgery is one of the risk factors for enhancement of pressure wave reflection in adult patients with congenital heart disease

BACKGROUND Excessive aortic pressure wave reflection is one of the risk factors of cardiovascular diseases. In some clinical course of congenital heart diseases, the elevated pressure wave reflection has been reported. The purpose of this study is to elucidate the risk factors of the enhanced pressure wave reflection in adult patients with congenital heart disease. METHODS We enrolled 99 adult (≥20 years) patients with congenital heart disease. We measured their radial pressure augmentation index and examined the relationship between it and various clinical variables. RESULTS The radial pressure augmentation index was 77.1 ± 19.1% and it had a significant correlation with the history of aorto-pulmonary shunt (t=4.194; p<0.0001), age (t=4.091; p<0.0001), height (t=-3.580; p=0.001) and the history of direct aortic surgery (t=2.253; p=0.027). Forty-four patients (44.4%) demonstrated high radial augmentation index (>1SD of age- and gender matched control) and the determinants of the elevated radial augmentation index were the history of aorto-pulmonary shunt (odds ratio, 21.32; 95% confidence interval, 5.47-83.14; p<0.0001) and the direct aortic surgery (4.18; 1.38-12.72; p=0.012). CONCLUSIONS The history of aortic surgeries enhances aortic pressure wave reflection in adult patients with congenital heart disease. The enhanced aortic pressure wave reflection is one of the risk factors for cardiovascular diseases. Therefore, the adult patients with congenital heart disease after aortic surgeries should be carefully observed and tightly controlled concerning the risk factors for cardiovascular diseases.

Efficacy and safety of tolvaptan for pediatric patients with congestive heart failure. Multicenter survey in the working group of the Japanese Society of PEdiatric Circulation and Hemodynamics (J-SPECH).

BACKGROUND Tolvaptan, a vasopressin V2-receptor antagonist, has been reported to improve congestion in adult patients with heart failure. However, it has not been fully clarified whether tolvaptan is also effective and safe for pediatric patients as well as adult. METHODS This trial was a multicenter, retrospective, observational study, and was led by the Japanese Society of PEdiatric Circulation and Hemodynamics (J-SPECH). Thirty-four pediatric patients who received tolvaptan to treat congestive heart failure were enrolled in this study. RESULTS An increment in the urinary volume and decrease in the body weight from baseline were significant at day 1 (+106.7 ± 241.5%, p = 0.008 and -2.30 ± 4.17%, p = 0.01), day 3 (+113.5 ± 261.9%, p = 0.02 and -2.30 ± 4.17%, p = 0.01), week 1 (+56.3 ± 163.5%, p = 0.01 and -1.55 ± 4.09%, p = 0.03) and month 1 (+91.1 ± 171.6%, p = 0.01 and -2.95 ± 5.98, p = 0.03). The significant predictive factors in responders, who was defined as patients who achieved an increase in the urinary volume at day 1, were older age (p = 0.03), larger body weight before exacerbation (p = 0.04), higher weight at one day before the first administration of tolvaptan (p = 0.03), higher aspartate aminotransferase levels (p = 0.03) and higher urinary osmolality levels (p = 0.03). A logistic regression analysis showed that the urinary osmolality was the only significant predictive factor for responders to tolvaptan. Adverse drug reactions were observed in 7 patients (20.6%). Six patients had thirst and a dry month, and 1 had a mild increase in the alanine aminotransferase and aspartate aminotransferase. CONCLUSION Tolvaptan can be effectively and safely administered in pediatric patients. Because the kidneys in neonates and infants are resistant to arginine vasopressin, the efficacy of tolvaptan may be less effective compared to older children.

Sheath placement in femoral artery during cardiac catheterization in children can influence pressure waveform

INTRODUCTION It is reported that pressure wave reflection is enhanced by external compression of the femoral artery. Therefore, it is possible that cardiac catheterization itself can influence the aortic pressure waveform. AIM The purpose of this study is to clarify the influence of sheath placement in a femoral artery on the pressure waveform. METHODS This study enrolled 21 pediatric patients (5.1±4.0years) who underwent cardiac catheterization. A sheath was placed in the femoral arteries of all patients. The change in the pressure waveform induced by the placement of the sheath was investigated using the b/a and d/a ratio of second derivative of a fingertip photoplethysmogram. A high b/a ratio means a stiff aorta and a low d/a ratio represents an enhancement of the aortic pressure wave reflection. RESULTS By the placement of the sheath in their femoral arteries, the b/a ratio was not influenced (sheath (-): -0.556±0.081 vs. sheath (+): -0.558±0.072; p=0.896). However, the d/a ratio was significantly decreased (-0.150±0.074 vs. -0.185±0.084; p=0.0003). CONCLUSIONS The placement of the femoral arterial sheath enhances the pressure wave reflection and would lead to a change in the central aortic pressure waveform.

Letter by Murakami and Niwa Regarding Article, “Unknown Complication of Arterial Switch Operation: Resistant Hypertension Induced by a Strong Aortic Arch Angulation”

We read with great interest the article by Ladouceur et al,1 which reported the enhancement of aortic pressure wave reflection in a patient after arterial switch procedure. They concluded that the shape of the aortic arch after the operation caused the enhancement of pressure wave reflection. Although the aortic morphology should be 1 of the causes of the enhancement, we speculate a different mechanism as well. It is well known that aortopathy frequently occurs in patients with congenital heart diseases.2 …

Impact of Pulmonary Valve Regurgitation on Pressure Difference of Pulmonary Valve Stenosis in Patients with Tetralogy of Fallot After Repair

Pressure difference (PD) is an important parameter in evaluating the degree of stenotic lesion. However, PD is influenced by the blood flow volume passing through the stenosis. In patients with tetralogy of Fallot (TOF), pulmonary valve regurgitation (PR) and pulmonary valve stenosis (vPS) are common post-operative complications. The aim of this study was to evaluate the influence of PR on the PD. First, we examined the relationship between the peak-to-peak PD and the valve orifice area in 7 patients with vPS from their cardiac catheterization data. Second, an estimated PD, i.e., PD assuming no PR, was calculated in 8 patients with TOF with vPS and PR from their valve orifice area using the relational equation in patients with vPS. Moreover, an excess of PD, equating to the difference between the measured and estimated PD, was calculated. Finally, the relationship between the regurgitant fraction (RF) and the excess PD was analyzed. There was a strong relationship between the reciprocal of the valve orifice area and the PD in patients with vPS (r = 0.904, p = 0.0053). The excess PD showed a significant correlation with the RF in patients with TOF (r = 0.889, p = 0.0032). PR of over 25% in RF augmented the PD depending on the regurgitant volume. Severity of vPS could be overestimated in post-operative patients with TOF who had significant PR when their RF was above 25%.

Body composition, appetite-related hormones, adipocytokines, and heart failure in adult patients with congenital heart disease: A preliminary study

OBJECTIVES To assess body composition and relationships among body composition, appetite-related hormones, adipocytokines, and heart failure (HF) in adult patients with congenital heart disease (CHD). PATIENTS This prospective study enrolled 46 consecutive adult patients with CHD and 12 age-matched healthy controls. The patients and control subjects were divided into four groups: 13 patients with Fontan circulation (group A), 16 patients with cyanosis (group B), 17 patients who previously underwent biventricular repair (group C), and 12 age-matched healthy controls. DESIGN Body composition was measured using InBody730, and levels of appetite-related hormones (ghrelin and leptin) and adipocytokines (leptin, interleukin-6, and tumor necrosis factor-α) were determined. Relationships of these measurements between severe HF, defined as New York Heart Association functional class III-IV and/or recent repeated unscheduled hospitalizations due to HF, were examined using univariate logistic analysis. RESULTS Mean patient age was 32.1 ± 7.4 years. The skeletal muscle mass was significantly decreased in groups A and B compared with that in controls. Interestingly, ghrelin levels in groups A and B were also significantly lower than those in controls. Univariate logistic analysis revealed that ghrelin level, percent body fat, and pulse oximetric oxygen saturation were significantly associated with severe HF. CONCLUSIONS Patients with Fontan circulation and those with cyanosis might be at a risk of sarcopenia. Despite the decreased skeletal muscle mass and increased body fat, ghrelin levels in these patients were decreased. These changes might have a negative impact on HF in these patients.

Letter by Murakami et al Regarding Article, “Systemic Right Ventricle in Adults with Congenital Heart Disease: Anatomic and Phenotypic Spectrum and Current Approach to Management”

We read with great interest the article by Brida et al1 that described an approach to the management of patients with systemic right ventricle (SRV). The article was highly informative; however, we would like to discuss pharmacological therapy in patients with SRV. In the article, the authors stated that β-blockers may have a beneficial …

Aortic Reservoir Function has a Strong Impact on the Cardiac Blood Supply–Workload Balance in Children

It has been reported that more than half of the ejected blood from the left ventricle is stored in the aorta during systole and expelled during diastole. One important organ that receives blood flow mainly during diastole is the heart. It is also reported that the cardiac blood supply–workload balance in small children is disadvantageous to the heart. Therefore, we measured the aortic reservoir function and examined the relationship between the aortic reservoir function and the cardiac blood supply–workload balance. The percent diastolic runoff, which is the percentage of the diastolic blood flow of the total cardiac output, was measured as the index of the aortic reservoir function. The subendocardial viability ratio—the ratio of the diastolic pressure time index (the blood supply to the heart) to the tension time index (implying the myocardial oxygen demand)—was investigated as an index of the cardiac blood supply–workload balance in children. The percent diastolic runoff was 51.7 ± 4.5%, smaller than that in adult. It had a significant positive relationship to age (r2 = 0.32, p = 0.0052). The subendocardial viability ratio was 100.8 ± 19.6% and had a strong relationship to the percent diastolic runoff (r2 = 0.92, p < 0.0001). The percent diastolic runoff had a positive relationship with age during childhood. The value had a strong impact on the cardiac blood supply–workload balance.

Pulse Wave Velocity and Augmentation Index

Pulse wave velocity is obtained from the transit time of pulse wave and the distance between two measuring sites. It is considered the “gold standard” for assessment of arterial elastance and recognized as one of the most important risk factors for cardiovascular diseases.

Letter by Murakami and Nawa Regarding Article, “Bilateral Superior Venae Cavae With Crisscross Atrial Drainage”

We read with great interest the article by Tripathi and colleagues1 that reported the anomalous connection of the right superior vena cava (RSVC) to the left atrium (LA) and the left superior vena cava (LSVC) draining into the right atrium via the coronary sinus in a patient with sick sinus syndrome. It is an extremely rare anomaly. The authors diagnosed the patient as having an “RSVC draining into the LA and persistent LSVC (PLSVC) …