Gardner Monique M, Cohen Meryl S
Division of Pediatric Cardiology, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA.
Congenit Heart Dis. 2017 Dec;12(6):783-786. doi: 10.1111/chd.12506. Epub 2017 Jun 23.
Noncompaction is a poorly understood form of cardiomyopathy that typically affects the left ventricle and may be associated with congenital heart disease. Right ventricular noncompaction (RVNC) may occur when the left ventricle is affected but is rarely seen in isolation. RVNC may have clinical significance affecting surgical and long-term outcomes. We describe the diagnosis and clinical course in three patients at our institution.
We performed a retrospective review of patients diagnosed with RVNC over a 12-month period at our institution and reviewed their imaging and clinical course.
Three patients were identified. All had diagnosis of RVNC by echocardiography (echo) made on postnatal imaging which reviewed degree of trabeculation, and noncompaction-to-compaction ratio of the myocardium. Patient A was a neonate with hypoplastic left heart syndrome (HLHS) who underwent a Norwood operation with Sano modification. Her postoperative course was notable for low-normal RV function. She returned with a pericardial effusion warranting immediate pericardiocentesis. She continued to have effusions, which were medically managed. She was subsequently found to have an RV apical pseudoaneurysm, which required surgical resection. Patient B was a neonate with HLHS who had a Norwood operation with Sano modification. She had low-normal RV function on echo. She required medical management for pericardial effusion. Patient C was a neonate with HLHS who also underwent a Norwood operation with Sano modification. His postoperative course was notable for elevated serum brain natriuretic peptide, which was treated with digoxin.
RVNC is a rare diagnosis with limited known clinical impact. One of these patients had a very rare complication after pericardiocentesis (pseudoaneurysm) that may have been related to the RVNC. Our understanding of this disease process is limited and requires additional investigation, but emphasizes the importance of appropriate diagnosis to allow for timely follow-up and counseling for this unique population.
心肌致密化不全是一种了解较少的心肌病形式，通常影响左心室，可能与先天性心脏病有关。当左心室受累时可能会发生右心室心肌致密化不全（RVNC），但孤立出现的情况很少见。RVNC可能具有临床意义，会影响手术和长期预后。我们描述了我院3例患者的诊断及临床病程。
我们对我院在12个月内诊断为RVNC的患者进行了回顾性研究，并回顾了他们的影像学和临床病程。
共确定了3例患者。所有患者均通过产后超声心动图（echo）诊断为RVNC，该检查评估了小梁化程度以及心肌致密化与非致密化的比例。患者A是一名患有左心发育不全综合征（HLHS）的新生儿，接受了改良诺伍德手术。她术后的病程特点是右心室功能略低于正常水平。她因心包积液复诊，需要立即进行心包穿刺术。她持续出现积液，通过药物治疗。随后发现她患有右心室心尖假性动脉瘤，需要手术切除。患者B是一名患有HLHS的新生儿，接受了改良诺伍德手术。超声心动图显示她的右心室功能略低于正常水平。她因心包积液需要药物治疗。患者C是一名患有HLHS的新生儿，也接受了改良诺伍德手术。他术后的病程特点是血清脑钠肽升高，通过地高辛治疗。
RVNC是一种罕见的诊断，已知的临床影响有限。其中一名患者在心包穿刺术后出现了一种非常罕见的并发症（假性动脉瘤），可能与RVNC有关。我们对这个疾病过程的了解有限，需要进一步研究，但强调了正确诊断对于为这一特殊人群进行及时随访和咨询的重要性。
