Configuration of the neoaortic root after chimney reconstruction in the Norwood procedure.

OBJECTIVES: After staged reconstruction for hypoplastic left heart syndrome, the neoaortic root tends to dilate, and the incidence of significant neoaortic valve insufficiency increases with time. This study aimed to evaluate the mid-term outcomes of the neoaortic root geometries and valve function after chimney reconstruction in the Norwood procedure. METHODS: Between 2013 and 2021, 20 consecutive patients who underwent chimney reconstruction during the Norwood procedure for hypoplastic left heart syndrome and its variants in our institution were enrolled. The actual diameters of the following points were measured, and Z-scores were calculated based on the normal aortic root geometries using the long axis view of echocardiography at the pre-Norwood stage and the lateral view of angiography at pre-Glenn, pre-Fontan, post-Fontan and follow-up (age 5-6 years) stages: neoaortic valve annulus; sinus of Valsalva; sinotubular junction; and ascending aorta just proximal to the anastomosis to the aortic arch. The degree of neoaortic valve regurgitation was evaluated by echocardiography at each stage. RESULTS: The median follow-up period was 3.9 years. Neoaortic roots after chimney reconstruction were spared from progressive dilation over time. With growth, the conical configuration of the neoaortic roots was preserved without geometrical distortion. The Z-scores of the annulus, sinus of Valsalva, sinotubular junction and ascending aorta ranged roughly from 4 to 6, 4 to 6, 2 to 4 and 0 to 2, respectively. All neoaortic valves at each stage had mild or no regurgitation. CONCLUSIONS: Chimney reconstruction prevented neoaortic root dilation and avoided significant neoaortic valve regurgitation in the mid-term. These neoaortic dimensions with smooth flow profiles in the neoaorta after chimney reconstruction may have contributed to the current results. Further studies are needed to clarify the long-term outcomes.

Effectiveness of cardiac palliative surgery for trisomy 18 patients with increased pulmonary blood flow.

Congenital heart disease (CHD) is common among patients with trisomy 18 (T18), but cardiac surgery has been rarely indicated for T18 patients due to their short life span. Although the therapeutic effects of aggressive interventions were recently demonstrated for T18 patients, the subjects and factors examined varied, resulting in inconsistent findings. Therefore, the effects of cardiac surgery for T18 remain unclear. We herein investigated the outcomes of cardiac palliative surgery for CHD with increased pulmonary blood flow in T18 patients. 27 patients were examined: 13 (48.1%) underwent cardiac palliative surgery and 14 (51.9%) did not. Median survival times in the no-surgery and surgery groups were 223.0 days (95% confidence interval [CI]: 46-361 days) and 723.0 days (95% CI: 360-1447 days), respectively. The number of patients with pulmonary hypertension significantly differed between the two groups (5 of 14 in the no-surgery group and 0 in the surgery group). Five of 14 patients in the no-surgery group and 10 of 13 in the surgery group were discharged to home care (odds ratio: 10.8 [95% CI: 1.07-110.0]). Therefore, cardiac palliative surgery may be used to treat CHD with increased pulmonary blood flow in T18 patients.

Biventricular repair using subvalvular techniques for unbalanced atrioventricular septal defect.

We report the case of a 19-month-old girl with a right-dominant unbalanced atrioventricular septal defect and severe right-sided atrioventricular valve regurgitation who underwent biventricular repair using basal chordae resection, artificial chordae reconstruction and a left-sided atrioventricular valvuloplasty. At 14-month postoperative follow-up, the patient had minimal heart failure, gained weight and adapted to biventricular circulation.

Veno-venous extracorporeal membrane oxygenation support of pulmonary insufficiency using Avalon Elite® cannula in pediatric patients.

The "Avalon Elite®" cannula is a double-lumen cannula used to establish veno-venous extracorporeal membrane oxygenation support. The reported advantages are that extracorporeal circulation can be established by cannulating through the right internal jugular vein only, and there is less re-circulation than with a two-cannula technique. It is available in a wide range of cannula sizes and can be used in a variety of patients, from children to adults. We herein report three pediatric cases in which an Avalon Elite® cannula was useful. The first was a case of acute mitral regurgitation due to idiopathic chordal rupture for postoperative severe lung injury and atelectasis due to cardiogenic pulmonary edema. The second was a case of end-stage radiation pneumonitis for safe transfer to facility of lung transplantation. The third was a convalescent case of fulminant myocarditis with severe atelectasis due to cardiogenic pulmonary edema. In each case, veno-venous extracorporeal membrane oxygenation using an Avalon Elite® cannula was established, the expected sufficient support was secured, and a good clinical course was obtained without major complications associated with an Avalon Elite® cannula.

A new predictor of the optimal pulmonary artery reconstruction method during the arterial switch operation.

OBJECTIVES: The aim of this study was to more accurately predict the optimal pulmonary artery (PA) reconstruction procedure (Lecompte manoeuvre or original Jatene procedure) during the arterial switch operation, we focused on the horizontal sectioning (HS) angle between the left hilum PA and the great arteries using preoperative computed tomography imaging. METHODS: We defined the HS angle α (ß) as the angle between the tangential line from the posterior (anterior) wall of the left PA at the hilum to the left anterior (right posterior) surface of the main PA and the tangential line from the left surface of the ascending aorta to the left anterior (right posterior) surface of the main PA. We identified 14 consecutive patients diagnosed with transposition of the great arteries or transposition of the great artery-type double-outlet right ventricle who underwent preoperative computed tomography imaging. The original Jatene or Lecompte procedure was used for 9 (OJ group) and 5 (L group) patients. Relationships of the great arteries of the OJ and L groups were side by side in 8 and 2 patients, oblique in 1 and 1 patient and anteroposterior in 0 and 2 patients, respectively. RESULTS: In the OJ group, ß was greater than α in all patients. The median α/ß value was 0.618. In group L, α was greater than ß in all patients. The median α/ß was 1.307. Left PA stenosis caused by stretching was not observed in the L group. Coronary obstruction was not identified in the OJ group. Left PA stenosis behind the neo-ascending aorta was observed in 1 patient in the OJ group and required reoperation. CONCLUSIONS: The HS angle may be a useful predictor of optimal intraoperative PA reconstruction during arterial switch operation, especially for side-by-side or oblique relationships.

Potential of the EVAHEART 2 Double-Cuff Tipless Inflow Cannula for Prevention of Thromboembolic Events.

A 32-year-old man, who had developed fulminant myocarditis leading to asystole, underwent implantation of an EVAHEART 2 left ventricular assist system with a double-cuff tipless inflow cannula and a concurrent Fontan operation. Approximately 2 years after the simultaneous EVAHEART 2 implantation and the Fontan operation, the patient underwent heart transplantation. There was no device-related thromboembolism or pump malfunction under adequate antithrombotic management during the postoperative support period. Computed tomography showed no malposition of the inflow cannula irrespective of the left ventricular chamber size. Macroscopically, the left ventricular cavity of the excised heart revealed a smooth inflow ostium with appropriate intimal proliferation and without pannus or wedge thrombus formation. These findings suggest the utility of the double-cuff tipless inflow cannula for long-term clinical applications, which may lead to favorable outcomes during long-term patient management. The double-cuff tipless inflow cannula, which does not protrude into the left ventricular cavity, potentially contributes to the prevention of suction events and the collision of the inflow cannula with the interventricular septum and left ventricular free wall. Further investigation is required to confirm the role of the unique EVAHEART 2 inflow cannula in reducing thromboembolic events.

Risk factors for discontinuation of exclusive breast feeding within 1month: a retrospective cohort study in Japan.

BACKGROUND: While breastfeeding provides benefits for infants and the mother, many women either do not breastfeed or terminate breastfeeding earlier than recommended. The aim of this analysis was to identify factors associated with early discontinuation of breastfeeding in Japanese women. METHODS: This study used data from medical records of women delivering a singleton live birth between March 2017 and August 2019 in Iwase General Hospital, Fukushima Prefecture, Japan to assess cessation of breastfeeding by the 1-month postpartum appointment. Demographic (age at birth, and employment status), medical (parity, and physical and mental condition of the mother; and infant medical factors, such as sex, Apgar score, and jaundice, among other), and family factors (husband/partner, family members living at the same house, among others) in 734 women who had initiated breastfeeding during their delivery hospital stay were examined, and multiple logistic regression was used to determine significant predictors of early cessation of exclusive breastfeeding. RESULTS: Bivariate analysis revealed that women who were primipara, unmarried, exposed to secondhand smoke, and employed; those who smoked before pregnancy; and those who had asthma were more likely to discontinue exclusive breastfeeding than other women. Infant factors associated with discontinuation were lower birthweight, earlier gestational age, neonatal intensive care unit admission, treatment for jaundice, or lower weight gain. Multivariable analysis revealed that primiparity, passive smoking before pregnancy, maternal employment, and neonatal jaundice therapy were associated with discontinuation of breastfeeding. CONCLUSIONS: In particular, women whose partners smoked before pregnancy may need to be targeted for additional support for breastfeeding.

Double Valve Replacement After Ventricular Assist Device Implantation in a Fontan Patient.

Few reports have described the implantation and management of an implantable ventricular assist device in patients with functional univentricular anatomy. We herein report a case of a patient who underwent Heartware ventricular assist device (Medtronic, Minneapolis, MN) implantation due to severe cardiac dysfunction and Fontan failure in the remote period after total cavopulmonary connection. Double valve replacement was subsequently required 1 year later due to repeated Fontan failure caused by the progression of aortic and atrioventricular regurgitation. The low-profile and saddle-shaped sewing cuff design of the MITRIS mitral valve (Edwards Lifesciences, Irvine, CA) were beneficial for avoiding the Heartware ventricular assist device inflow and atrioventricular valve interference in the less-dilated ventricle.

Valve-Sparing Reimplantation for Right Coronary Artery Compression After Fontan Procedure.

An incidental finding of right coronary artery compression between the dilated aortic root and the sternum was detected on follow-up cardiac catheterization after Fontan completion for tricuspid atresia with transposition of the great arteries. Although this 31-year-old man had no subjective symptoms, an exercise stress test and perfusion scan revealed ischemic changes in the inferior region. Valve-sparing aortic root replacement using the reimplantation technique proved to be an effective approach for this condition. The possibility of coronary artery compression associated with aortic root dilatation should be considered in adult patients with congenital heart disease.

Outcomes of systemic-to-pulmonary artery shunt for single ventricular heart with extracardiac total anomalous pulmonary venous connection.

OBJECTIVES: A few studies have described the outcomes of systemic-to-pulmonary artery shunt (SPS) for functional single ventricular heart with extracardiac total anomalous pulmonary venous connection (TAPVC). This study aimed to examine the outcomes of SPS with extracardiac TAPVC and identify the predictors of mortality before bidirectional Glenn operation (BDG). METHODS: Medical records of 41 children with single ventricular heart and extracardiac TAPVC who underwent SPS between 1998 and 2019 were reviewed retrospectively. The median age and body weight at SPS were 36 days and 3.4 kg, respectively. Surgical outcomes and predictors of mortality were investigated. RESULTS: Four operative deaths (10%) and 10 late deaths (27%) occurred before BDG. Of all the children, 19 underwent BDG at a median of 10 months since SPS and eight are waiting for BDG. In the multivariate analysis, preoperative pulmonary venous obstruction (p = 0.01) at initial surgery was most predictive of death before BDG. Patients who underwent simultaneous SPS and TAPVC repair were younger, had high preoperative rate of pulmonary venous obstruction, and more deaths before BDG. CONCLUSIONS: Survival outcomes of SPS for SVH with extracardiac TAPVC were improved as a whole due to the increase in knowledge and technique of management SPS. However, the patients who have preoperative pulmonary venous obstruction (PVO) and need SPS and TAPVC repair concomitantly in the early postnatal period have poor outcomes and still challenging. In such a case, staged TAPVC repair and SPS may be beneficial.

[Management of Functionally Univentricular Heart with Heterotaxy].

Various pathologies of heterotaxy should be diagnosed by documenting the arrangement of the cardiac structures and other organs. They carry functional derangements of cardiovascular systems and abdominal organs, such as atrioventricular valve dysfunction, pulmonary vein obstruction, arrhythmia, and intestinal malrotation. An appropriately organized management of these malformations is pivotal for improving outcomes.

Systemic-to-Pulmonary Collateral Flow Correlates with Clinical Condition Late After the Fontan Procedure.

In the Fontan circulation, there is a substantial degree of systemic-to-pulmonary collateral flow (SPCF), which can be measured by cardiac magnetic resonance (CMR). However, the correlation between the degree of SPCF and long-term outcomes is not fully understood. We retrospectively studied 321 patients who underwent the Fontan procedure and CMR at a single center. Using CMR, we calculated SPCF as pulmonary blood flow - systemic blood flow. %SPCF was defined as SPCF ÷ pulmonary blood flow. The mean age of patients at CMR was 14.3 ± 7.5 years. The average %SPCF was 13.0% ± 11.0%. With a multivariate analysis, %SPCF was significantly correlated with time (i.e., the longer the time period since the Fontan procedure, the lower the %SPCF) (p = 0.006), previous total anomalous pulmonary vein drainage (p = 0.007), a low pulmonary artery index (Nakata index) before the Fontan procedure (p = 0.04), and older age at the time of the Fontan procedure (p = 0.002). Regarding the findings after the Fontan procedure, %SPCF was significantly correlated with ventricular end-diastolic volume (p < 0.001), ventricular end-systolic volume (p < 0.001), central venous pressure (p < 0.001), plasma brain natriuretic peptide concentration (p < 0.001), hemoptysis (p = 0.009), and poor New York Heart Association functional class (p = 0.007). SPCF was correlated with clinical condition after the Fontan procedure. The importance of sufficient growth of the pulmonary vascular bed should be emphasized because the development of SPCF is believed to result from the poor condition of the pulmonary circulation.

Expansion of a Huge Compressive Left Atrial Appendage Aneurysm in a 29-Day-Old Infant.

We report a case of symptomatic and progressive enlargement of a huge left atrial appendage aneurysm in a 29-day-old infant. The aneurysm was detected by fetal echocardiography and exertional dyspnea developed during the neonatal period. The aneurysm was successfully resected by decompressing the aneurysm using cardiopulmonary bypass without cardiac arrest. Our findings suggest that fetal echocardiography enables early diagnosis of the rare left atrial appendage aneurysm, and early surgical resection may protect affected patients from life-threatening symptoms.

Outcomes of mitral valve replacement with bileaflet mechanical prosthetic valve in children.

OBJECTIVES: We examined the outcomes following mitral valve replacement with bileaflet mechanical prosthetic valve in children and identified the predictors for mortality and reoperation. METHODS: Medical records from 49 children who underwent mitral valve replacement between 1982 and 2015 were reviewed retrospectively. Median age and body weight at initial mitral valve replacement were 2.4 years and 9.7 kg, respectively. The median follow-up was 13 years. Surgical results and predictors for mortality and reoperation were investigated. RESULTS: There was no operative mortality; eight late deaths occurred. The actuarial survival rates were 89.5%, 84.2%, and 80.7% at 5, 10, and 15 years, respectively, after initial mitral valve replacement. The actuarial freedom rates from related complications were 89.5%, 78.3%, and 70.7% at 5, 10, and 15 years, respectively. Nineteen patients required 1st re-mitral valve replacement at a median of 5.9 years; six of these 19 required 2nd re-mitral valve replacement at a median of 8.9 years after 1st re-MVR. The actuarial freedom rates from re-mitral valve replacement were 86.0%, 56.8%, and 44.2% at 5, 10, and 15 years, respectively. No predictor for death was determined; however, the predictor for re-mitral valve replacement was initial valve diameter less than 19 mm. CONCLUSIONS: Survival outcomes among children after mitral valve replacement with bileaflet mechanical prosthetic valve in biventricular heart were satisfactory. However, complications, including re-mitral valve replacement, were frequent and the predictor was of a small prosthesis size.

[Evaluation for Resolution Property of CT Image with Half-reconstruction Algorithm].

PURPOSE: The aim of this study was to investigate the resolution property in XY-plane for half-reconstruction computed tomography (CT) image by measuring 360° multi-directional modulation transfer functions (MTFs). MATERIALS AND METHODS: The 360° multi-directional MTFs were measured by use of a wire method to obtain line spread function with 15° interval in XY-plane. The MTFs of half-reconstruction CT image were measured with 100 mm off-center positions on the X- and Y-axis (X+100 mm, X-100 mm, Y+100 mm, and Y-100 mm) and compared with those of full-reconstruction CT image. We measured the MTFs of the half-reconstruction CT image at X+100 mm position with various X-ray tube positions of projection dataset. RESULTS: There were obvious differences for the MTFs of the half-reconstruction CT image between the tangential and radial directions at each measurement position. The dependences of the resolution property for the half-reconstruction CT image on positions and directions in XYplane were similar to those for the full-reconstruction CT image. The higher and the lower MTFs of the half-reconstruction CT image at X+100 mm position were measured with X-ray tube position of projection dataset at +X side and at -X side compared with those of the full-reconstruction CT image, respectively. CONCLUSION: We conclude that the half-reconstruction CT image had similar resolution property in XY-plane to the full-reconstruction CT image and showed dependency on the X-ray tube position of projection dataset for MTF in the tangential direction.

Surgical results of total anomalous pulmonary venous connection repair in 256 patients.

OBJECTIVES: This study was performed to analyse the surgical results of total anomalous pulmonary venous connection (TAPVC) repair at a single institution and to identify trends and variables associated with mortality and morbidity, particularly predictors of recurrent pulmonary venous obstruction (PVO). METHODS: Our surgical database contained 256 patients with biventricular anatomy who underwent surgical repair for TAPVC from 1981 to 2016. The anatomic TAPVC subtypes in this study were as follows: 114 supracardiac (44.5%), 56 cardiac (21.9%), 64 infracardiac (25.0%) and 22 mixed (8.6%) types. The follow-up for the entire study ranged from 1.6 months to 28.2 years (median 10.4 years). Preoperative PVO was present in 128 patients (50.0%). All patients with TAPVC with single-ventricle anatomy were excluded from the analysis. Data were retrospectively reviewed. RESULTS: Seven (2.7%) early deaths and 26 (10.1%) late deaths occurred. The actuarial survival rate at 20 years postoperatively was 85.3%. The preoperative predictors of operative mortality were younger age and the era of TAPVC repair (before 1998). In addition to these variables, associated cardiac anomalies were predictors of late mortality. Those for postoperative PVO were younger age, lower weight and being an emergency case. The actuarial survival rate at 20 years was 38.6% for patients with postoperative PVO and 92.2% for patients without postoperative PVO (P < 0.001). CONCLUSIONS: The long-term outcomes after TAPVC repair in patients with biventricular anatomy were satisfactory. Mortality was significantly associated with the rate of progression of postoperative PVO, and careful follow-up was required especially within 6 months after the primary operation.

Long-Term Growth of the Neoaortic Root After Arterial Switch Operation.

BACKGROUND: The growth of the neoaortic root after the arterial switch operation for the transposition of the great arteries remains unclear. This study aimed to investigate the growth of the neoaortic root and identify risk factors for neoaortic root dilatation. METHODS: Serial angiographic measurements of the neoaortic root for at least 10 years were evaluated in 145 patients. A total of 1,876 measurements of the sinuses of the Valsalva and the neoaortic annuli were obtained. A linear mixed effects model was used for z-score analysis, including evaluation of risk factors for neoaortic root dilatation. To assess changes in the time course of neoaortic root absolute diameters, a nonlinear mixed effects model with a growth curve model was used. RESULTS: The growth curve revealed progressive growth of the neoaortic root during somatic growth and stabilization in adulthood without normalization. The growth rates of the sinus and annulus were 0.0046 and 0.029 z-score per year, respectively. The sinus and annulus were estimated to grow up to 47 ± 1 mm and 31 ± 1 mm, respectively. Major risk factors for neoaortic root dilatation were double-outlet right ventricle (parameter estimate [PE] = 2.1, 95% confidence interval [CI] = 1.5 to 2.7, p < 0.0001 for sinus; PE = 1.2; 95% CI = 0.7 to 1.6, p < 0.0001 for annulus) and presence of neoaortic valve insufficiency (PE = 0.9; 95% CI = 0.4 to 1.5; p < 0.001 for sinus; PE = 1.6, 95% CI = 1.2 to 2.0, p < 0.0001 for annulus). CONCLUSIONS: The risk for neoaortic root dilatation was common. Long-term surveillance is mandatory, particularly in patients with double-outlet right ventricle and neoaortic valve insufficiency.

Impact of age at bidirectional cavopulmonary anastomosis on haemodynamics after Fontan operation.

BackgroundsThe aim of this study was to assess the impact of age at bidirectional cavopulmonary anastomosis on haemodynamics after total cavopulmonary connection. METHODS: We conducted a retrospective analysis of 100 consecutive patients who underwent total cavopulmonary connection from 2010 to 2014. All patients had previously undergone bidirectional cavopulmonary anastomosis. These patients were classified into two groups according to age at bidirectional cavopulmonary anastomosis: younger group, 6 months (n=67). RESULTS: The proportion of hypoplastic left heart syndrome was higher in the younger group (48 versus 4%). After total cavopulmonary connection, the chest tube period was longer in the younger group (10.1±6.6 versus 6.7±4.5 days; p=0.009). Catheterisation 6 months after total cavopulmonary connection revealed that pulmonary artery pressure was higher (11.5±1.9 versus 10.4±2.1 mmHg; p=0.017) and Nakata index was lower (219±79 versus 256±70 mm2/m2; p=0.024) in the younger group. In patients with a non-hypoplastic left heart syndrome, there was no difference in post-operative haemodynamics between two groups, but the total amount of chest drainage after total cavopulmonary connection was larger in the younger group (109±95 versus 55±40 ml/kg; p=0.044). CONCLUSIONS: Early bidirectional cavopulmonary anastomosis did not affect the outcome of total cavopulmonary connection. Longer chest tube period, smaller pulmonary artery, and higher pulmonary artery pressure after total cavopulmonary connection were recognised in early bidirectional cavopulmonary anastomosis patients, especially in hypoplastic left heart syndrome.

Yasui Conversion for Repair After Left Ventricular Outflow Tract Obstruction.

We herein report two cases of progressive left ventricular outflow obstruction after primary repair of arch obstruction and ventricular septal defect that was successfully resolved with Yasui conversion. Patients who require surgical reintervention for progressive left ventricular outflow tract (LVOT) obstruction after primary biventricular repair of interruption of the aortic arch or coarctation of the aorta complex are occasionally experienced. The modified Konno procedure and Ross operation are well recognized as useful for these cases. However, in some patients, these procedures are difficult to perform because of anatomic restrictions or previous procedures. Although the indications are limited, the Yasui conversion is a safe, simple, and useful option for LVOT obstruction after primary biventricular repair.