Use of the index of pulmonary vascular disease for predicting long-term outcome of pulmonary arterial hypertension associated with congenital heart disease.

Aims: Limited data exist on risk factors for the long-term outcome of pulmonary arterial hypertension (PAH) associated with congenital heart disease (CHD-PAH). We focused on the index of pulmonary vascular disease (IPVD), an assessment system for pulmonary artery pathology specimens. The IPVD classifies pulmonary vascular lesions into four categories based on severity: (1) no intimal thickening, (2) cellular thickening of the intima, (3) fibrous thickening of the intima, and (4) destruction of the tunica media, with the overall grade expressed as an additive mean of these scores. This study aimed to investigate the relationship between IPVD and the long-term outcome of CHD-PAH. Methods: This retrospective study examined lung pathology images of 764 patients with CHD-PAH aged <20 years whose lung specimens were submitted to the Japanese Research Institute of Pulmonary Vasculature for pulmonary pathological review between 2001 and 2020. Clinical information was collected retrospectively by each attending physician. The primary endpoint was cardiovascular death. Results: The 5-year, 10-year, 15-year, and 20-year cardiovascular death-free survival rates for all patients were 92.0%, 90.4%, 87.3%, and 86.1%, respectively. The group with an IPVD of ≥2.0 had significantly poorer survival than the group with an IPVD <2.0 (P = .037). The Cox proportional hazards model adjusted for the presence of congenital anomaly syndromes associated with pulmonary hypertension, and age at lung biopsy showed similar results (hazard ratio 4.46; 95% confidence interval: 1.45-13.73; P = .009). Conclusions: The IPVD scoring system is useful for predicting the long-term outcome of CHD-PAH. For patients with an IPVD of ≥2.0, treatment strategies, including choosing palliative procedures such as pulmonary artery banding to restrict pulmonary blood flow and postponement of intracardiac repair, should be more carefully considered.

[The Apical Left Ventricle Aneurysm, Eighteen Years After the Fulminant Myocarditis:Report of a Case].

Ventricular aneurysm after myocarditis is a rare complication. It has been reported that scarred areas of the myocardium may become aneurysm. Here, we report a case of apical left ventricle aneurysm at 18 years after the fulminant myocarditis. The patient is a 36-year-old female with a history of fulminant myocarditis at the age of 18. Eighteen years after the onset of the disease, she was referred to our hospital because of an apex ventricular aneurysm. Her computed tomography scan showed an apex ventricular aneurysm sized 45×40 mm with a wall width of 1.8 mm. We performed resection of ventricular aneurysm and reconstruction using Linear technique. The postoperative course was uneventful and discharged on the 17th post operative day.

Spontaneous splenic rupture, mesenteric ischemia and spinal infarction after aortic repair for acute type A dissection in a patient with sickle cell trait.

Sickle cell trait (SCT), a benign hematological condition affecting approximately 300 million individuals globally, is associated with an increased risk of vaso-occlusive disease. However, the risks related to surgery employing cardiopulmonary bypass in patients with SCT are not well established. Herein, we report the case of a 27-year-old African American man with SCT who underwent an emergency aortic repair for acute Stanford type A aortic dissection using hypothermic circulatory arrest. The patient developed a sickle cell crisis, which was followed by spontaneous splenic infarction and rupture, nonocclusive mesenteric ischemia, and spinal infarction.

[Rhabdomyolysis during Perioperative Period after Cardiac Surgery].

We report a case of rhabdomyolysis during a perioperative period after cardiac surgery. A 47-yearold man underwent aortic root replacement for annuloaortic ectasia under general anesthesia using sevoflurane, fentanyl, remifentanil, rocronium bromide and midazolam. On the 1st postoperative day (1 POD), his body temperature rose over 38 ℃, which continued for 3 days despite our attempt to stabilize the fever. On 4 POD, his laboratory data and hemodynamics dramatically worsened, and we commenced continuous hemodialysis filtration (CHDF) and percutaneous cardiopulmonary support system (PCPS). In addition, balloon pumping (IABP) was started on 5 POD. At the same time, we initiated dantrolene sodium hydrate infusion according to a clinical grading scale to predict malignant hyperthermia (MH) susceptibility. Serum creatine phosphokinase (CPK) increased over 350,000 U/l on the 7 POD, and dantrolene sodium hydrate was continuously infused until 9 POD. Despite dantrolene sodium infusion, CHDF, IABP and PCPS, his condition did not improve, and he died of disseminated intravascular coagulation syndrome (DIC) and sepsis on 28 POD. Computed tomography on 21 POD disclosed scattered low-density areas in the erector spinal, lliopsoas and femoral muscles, which indicated rhabdomyolysis. Histopathological examination using hematoxylin and eosin stain revealed destroyed striated-muscle fibers and swelling rhabdomyocytes. It remained unclear which drug triggered rhabdomyolysis. When MH is suspected, we should consider the use of the clinical grading scale to predict its susceptibility and start dantrolene sodium hydrate infusion.

Norwood Procedure Performed on a Patient With Trisomy 13.

Trisomy 13 is associated with a variety of congenital anomalies, some of which are life-threatening and related to poor prognosis. Therefore, cardiac surgery is rarely offered to these patients, especially to those with complex cardiac anomalies. We report the case of a neonate weighing 2324 g who was born with severe congenital heart defects. Transthoracic echocardiography revealed the diagnoses of asplenia, single ventricle, aortic stenosis, coarctation of the aorta, hypoplastic aortic arch, and total anomalous pulmonary venous return. She was hemodynamically unstable. Palliative Norwood procedure with right ventricle-pulmonary artery conduit (RV-PA conduit) was performed at the age of 1 day to save her life. On postoperative day 7, chromosome analysis revealed trisomy 13. Echocardiography revealed good heart function; stable hemodynamic status was achieved with minimal amounts of inotropic agents. However, she developed anuria, which did not improve despite situational possible interventions, including peritoneal dialysis and continuous hemodiafiltration. On postoperative day 37, she succumbed to sudden cardiorespiratory failure. Nevertheless, this case indicates that a neonate with trisomy 13 can have a better chance at survival with cardiac surgery such as the Norwood procedure with an RV-PA conduit.

Impact of the entry site on late outcome in acute Stanford type B aortic dissection†.

OBJECTIVES: This study aimed to investigate whether the entry site of acute type B aortic dissection affects late outcomes. METHODS: Inpatient and outpatient records were retrospectively reviewed. RESULTS: We identified 224 cases of acute type B aortic dissection between 1998 and 2013. Of these 224 patients, 168 were men and the age was 64.2 ± 12.6 (range 23-94) years, from which 130 presented with the entry at a location downstream of the distal aortic arch, 67 with the entry at the outer curvature of the distal aortic arch and 27 with the entry at the inner curvature. At the initial presentation, 127 patients had descending false lumen thrombosis. The 30-day mortality rate was 2%, and 8% of patients had malperfusion. The entry at the outer curvature was associated with a higher risk of 30-day mortality. Patients with the entry at a location downstream were significantly older, and had a higher chance for primarily thrombosed descending false lumen and a lower risk of malperfusion. At follow-up (6.0 ± 4.1 years), the actuarial survival rates were 97, 83 and 60%, freedoms from open aortic surgery were 96, 91 and 86%, aortic intervention were 73, 66 and 63% and aortic events were 71, 60 and 52% at 1, 5 and 10 years, respectively. Multivariate logistic regression analysis revealed that the outer curvature entry and maximum aortic diameter were correlated with open aortic surgery, aortic intervention and aortic events. Of the 127 patients with primarily thrombosed false lumen, the outer curvature entry was significantly correlated with aortic events. CONCLUSIONS: The primary entry at the outer curvature of the distal aortic arch, as well as the large aortic diameter, is associated with a higher risk of late open aortic surgery, aortic intervention and aortic events in acute type B aortic dissection. Thus, the entry site should be taken into consideration in the establishment of an appropriate treatment indication of type B aortic dissection.