Amula Venugopal, Ellsworth German L, Bratton Susan L, Arrington Cammon B, Witte Madolin K
Pediatr Cardiol. 2014 Jan;35(1):38-46. doi: 10.1007/s00246-013-0736-y.
Patients with heterotaxy syndrome (HS) have significant cardiac and extracardiac anomalies that impact outcome. To improve the management of this complex patient population, we performed a comprehensive analysis of their anatomic and clinical features along with an evaluation of resource utilization data. The objectives were to describe anatomic and clinical features of patients with HS syndrome treated at a single center from 1992 to 2011 focusing on the impact of ventricular morphology (univentricular [UV] vs. biventricular [BV]) on clinical outcomes and resource utilization. Clinical and echocardiographic data from patients with HS were abstracted from medical records. Health care costs were indexed to inflation. Seventy-eight patients were identified with HS ranging in age from 1 day to 29 years old. UV morphology was present in 46 patients (59 %), most commonly with right-ventricular dominance (36 of 46). The presence of extra cardiac anomalies did not differ between the UV and BV groups (82 vs. 78 %) nor did morbidities, such as need for enteral tube feedings (47 vs. 25 %) or pacemaker placement (24 vs. 25 %). Mortality was 28 % in the entire cohort: 39 % in univentricuar patients versus 10.5 % in those with biventricular anatomy. Hospital length of stay for medical illnesses was similar in both groups, but length of stay after surgery was significantly longer in UV than BV patients. Among survivors, UV patients had greater median hospital costs (TeX 67,732, p < 0.001), but when this was adjusted for mortality and variable follow-up, there were no differences in health care costs within the first year of life. Significant health care dollars are used to manage children with HS, the majority of which involve expenses related to surgical care. Although patients with biventricular morphology have better survival, morbidity and resource utilization are similar to those for UV patients especially within the first year of life.
内脏异位综合征（HS）患者存在显著的心脏和心外异常，这些异常会影响预后。为改善对这一复杂患者群体的管理，我们对他们的解剖学和临床特征进行了全面分析，并评估了资源利用数据。目的是描述1992年至2011年在单一中心接受治疗的HS综合征患者的解剖学和临床特征，重点关注心室形态（单心室[UV]与双心室[BV]）对临床结局和资源利用的影响。HS患者的临床和超声心动图数据从病历中提取。医疗保健成本根据通货膨胀进行了指数化调整。共确定了78例HS患者，年龄从1天至29岁不等。46例患者（59%）存在UV形态，最常见的是右心室优势（46例中的36例）。心脏外异常的存在在UV组和BV组之间无差异（82%对78%），发病率也无差异，如肠内管饲需求（47%对25%）或起搏器植入（24%对25%）。整个队列的死亡率为28%：单心室患者为39%，而双心室解剖结构患者为10.5%。两组因内科疾病的住院时间相似，但UV患者术后住院时间明显长于BV患者。在幸存者中，UV患者的中位住院费用更高（67,732美元，p<0.001），但在对死亡率和可变随访进行调整后，生命第一年的医疗保健成本无差异。大量医疗保健资金用于管理HS患儿，其中大部分涉及与手术治疗相关的费用。尽管双心室形态的患者生存率更高，但发病率和资源利用与UV患者相似，尤其是在生命的第一年。
